Definition Causes / risk factors

MIND MAPS FOR MEDICINE

Symptoms

MIND MAPS FOR MEDICINE

An innovative, visual textbook to help medical students learn
and understand core medical conditions. Signs

The book features over 100 easy to follow, All mind maps are presented consistently
full colour mind maps of clinically relevant and cover: Pathophysiology
medical conditions using a systems-based • Definition Clinical features
structure: • Pathophysiology
• Cardiology • Causes and risk factors
• Respiratory • Clinical features: signs and symptoms
• Gastroenterology • Epidemiology
• Renal • Investigations: blood tests and imaging
• Endocrinology • Management: lifestyle, pharmacological
• Neurology and surgical
• Rheumatology • Complications
• Infectious diseases

MIND MAPS
Other key features:
The mind maps give you quick access to • Images are provided throughout the
key information in a visually appealing book to help illustrate key signs.
way. Where appropriate the mind map • Mnemonics are used throughout to
is followed by additional reference aid learning.

FOR MEDICINE
information to remind you about, for • Information is up-to-date and based
example, risk assessment tools, staging around the latest guidelines.
criteria, and treatment algorithms. • All topics are clinically relevant or likely to
appear in medical school examinations.
Mind Maps for Medicine is crucial reading for all medical students
but particularly those who consider themselves visual learners.

Pre-publication reviews from medical students

“Exactly what has been missing from the medical school textbook scene. It encompasses all
the need-to-know conditions, in a succinct yet detailed way, which makes studying easy.”
Blood tests
“This great book utilises the mind map format to present the core information, with handy
revision aids throughout!” Management

Azam
“Concise, colourful, and easy-to-follow – a great guide to common medical pathologies.” Investigations
“The mind maps are great – easy to follow with just the right amount of detail for medical students.”
Pharmacological
“The level of detail, organisation of information and layout are great and will make studying very
Imaging
enjoyable!! I think the addition of the ‘notes’ areas for many of the conditions is a great feature too!”
Lifestyle Surgical
“These mind maps look great! Absolutely nailed it in terms of level of detail and layout/design”

www.scionpublishing.com
ISBN 978-1-911510-36-9
Complications
9 781911 510369
Mohsin Azam

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 MIND MAPS
FOR MEDICINE
 MIND MAPS
Student reviewers
Our medical textbooks are assessed and reviewed by the following medical students:

University of Aberdeen School of Medicine and Dentistry: Dylan McClurg

FOR MEDICINE
Barts and The London School of Medicine and Dentistry: Jay Singh
University of Birmingham College of Medical and Dental Sciences: Hannah Morgan
Cardiff University School of Medicine: Chloe Chia
The University of Edinburgh Medical School: Tanith Bain
University of Exeter Medical School: Zoe Foster
Hull York Medical School: Maalik Imtiaz
King’s College London GKT School of Medical Education: Karan Sagoo
University of Liverpool School of Medicine: Sophie Gunter
University of Manchester Medical School: Holly Egan
Newcastle University School of Medical Education: Jenita Jona James
Norwich Medical School: Aneesa Khan
University of Nottingham School of Medicine: Tom Charles
University of Sheffield Medical School: Rebecca Nutt
St George’s, University of London: Nawshin Basit
Swansea University Medical School: Jack Bartlett
University of Central Lancashire School of Medicine: Katie Chi Kei Cheung
University College London Medical School: Camila Nicklewicz
We are grateful for their essential feedback.
Mohsin Azam, MBChB, BSc, MRCGP
Additional student reviewers wanted
We are keen to recruit more student reviewers, particularly at UK medical schools where we don’t
currently have anyone in post. If you would like to apply for the position, please contact Simon Watkins
(simon.watkins@scionpublishing.com) and explain why you would be suitable for the role. General Practitioner, North London

Feedback, errors and omissions

We are always pleased to receive feedback (good and bad) about our books – if you would like to comment on
any of our books, please email info@scionpublishing.com.

We’ve worked really hard with the authors to ensure that everything in the book is correct. However, errors
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future printings and post a note about it on our website so that other readers of the book are alerted to this.

Thank you for your help.

© Scion Publishing Ltd, 2021

First published 2021

All rights reserved. No part of this book may be reproduced or transmitted, in any form or by
any means, without permission.

A CIP catalogue record for this book is available from the British Library.

Contents
ISBN 9781911510369

Scion Publishing Limited

The Old Hayloft, Vantage Business Park, Bloxham Road, Banbury OX16 9UX, UK

www.scionpublishing.com Preface...............................................................................................................................................................ix
Acknowledgements ................................................................................................................................. x
Important Note from the Publisher Dedications .................................................................................................................................................... x
The information contained within this book was obtained by Scion Publishing Ltd Abbreviations................................................................................................................................................xi
from sources believed by us to be reliable. However, while every effort has been made
to ensure its accuracy, no responsibility for loss or injury whatsoever occasioned to
any person acting or refraining from action as a result of information contained herein
Chapter 1: Cardiology 1
can be accepted by the authors or publishers.

Readers are reminded that medicine is a constantly evolving science and while the Angina pectoris............................................................................................................................................ 2
authors and publishers have ensured that all dosages, applications and practices Acute coronary syndrome..................................................................................................................... 4
are based on current indications, there may be specific practices which differ
Acute pericarditis......................................................................................................................................... 8
between communities. You should always follow the guidelines laid down by the
manufacturers of specific products and the relevant authorities in the country in Atrial fibrillation......................................................................................................................................... 10
which you are practising. Valvular heart disease............................................................................................................................ 12
Although every effort has been made to ensure that all owners of copyright material Heart failure.................................................................................................................................................. 14
have been acknowledged in this publication, we would be pleased to acknowledge in Hypertrophic obstructive cardiomyopathy............................................................................. 18
subsequent reprints or editions any omissions brought to our attention. Hypertension.............................................................................................................................................. 20
Registered names, trademarks, etc. used in this book, even when not marked as such, Infective endocarditis............................................................................................................................ 24
are not to be considered unprotected by law.

Chapter 2: Respiratory 27

Acute respiratory distress syndrome............................................................................................ 28

Asthma............................................................................................................................................................ 30
Bronchiectasis............................................................................................................................................. 34
Chronic obstructive pulmonary disease.................................................................................... 36
Cystic fibrosis............................................................................................................................................... 40
Interstitial lung disease......................................................................................................................... 42
Lung cancer................................................................................................................................................. 46
Obstructive sleep apnoea................................................................................................................... 50
Pleural effusion.......................................................................................................................................... 52
Pneumonia................................................................................................................................................... 54
Pneumothorax........................................................................................................................................... 56
Typeset by Medlar Publishing Solutions Pvt Ltd, India
Pulmonary embolism............................................................................................................................ 60
Printed in the UK Respiratory failure.................................................................................................................................... 62
Last digit is the print number: 10 9 8 7 6 5 4 3 2 1 Sarcoidosis.................................................................................................................................................... 64
Mind Maps for Medicine \ v
 Chapter 3: Gastroenterology 67
Hypoglycaemia........................................................................................................................................168
Hyponatraemia........................................................................................................................................170
Acute liver failure...................................................................................................................................... 68 Hypopituitarism......................................................................................................................................174
Acute pancreatitis.................................................................................................................................... 70 Phaeochromocytoma..........................................................................................................................176
Alpha-1 antitrypsin deficiency......................................................................................................... 72 Polycystic ovary syndrome...............................................................................................................178
Chronic pancreatitis................................................................................................................................ 74
Cirrhosis.......................................................................................................................................................... 76 Chapter 6: Neurology 181
Coeliac disease........................................................................................................................................... 80
Bell’s palsy....................................................................................................................................................182
Crohn’s disease........................................................................................................................................... 82
Delirium........................................................................................................................................................186
Gastric cancer............................................................................................................................................. 86
Epilepsy.........................................................................................................................................................190
Gastro-oesophageal reflux disease............................................................................................... 88
Extradural haematoma (epidural haemorrhage)................................................................194
Hereditary haemochromatosis........................................................................................................ 90
Guillain–Barré syndrome...................................................................................................................196
Irritable bowel syndrome.................................................................................................................... 92
Migraine and other causes of headache.................................................................................198
Jaundice......................................................................................................................................................... 94
Motor neurone disease.......................................................................................................................202
Oesophageal cancer and other causes of dysphagia........................................................ 96
Multiple sclerosis....................................................................................................................................204
Peptic ulcer disease...............................................................................................................................100
Myasthenia gravis...................................................................................................................................206
Primary biliary cholangitis.................................................................................................................102
Neurofibromatosis.................................................................................................................................208
Primary sclerosing cholangitis........................................................................................................104
Parkinsonism.............................................................................................................................................210
Ulcerative colitis......................................................................................................................................106
Polyneuropathies....................................................................................................................................214
Upper gastrointestinal bleed..........................................................................................................108
Transient ischaemic attack................................................................................................................216
Wilson’s disease.......................................................................................................................................112
Stroke.............................................................................................................................................................218
Chapter 4: Renal 115
Subarachnoid haemorrhage...........................................................................................................222
Subdural haematoma..........................................................................................................................224
Acute kidney injury...............................................................................................................................116
Chronic kidney disease.......................................................................................................................118 Chapter 7: Rheumatology 227
Nephritic syndrome..............................................................................................................................122
Ankylosing spondylitis........................................................................................................................228
Nephrotic syndrome............................................................................................................................126
Fibromyalgia..............................................................................................................................................230
Urinary tract infection..........................................................................................................................128
Giant cell arteritis....................................................................................................................................232
Chapter 5: Endocrinology 131
Gout................................................................................................................................................................234
Osteoarthritis.............................................................................................................................................236
Acromegaly................................................................................................................................................132 Osteoporosis..............................................................................................................................................238
Adrenal insufficiency............................................................................................................................134 Paget’s disease.........................................................................................................................................240
Cushing syndrome................................................................................................................................136 Polymyalgia rheumatica.....................................................................................................................242
Diabetes insipidus..................................................................................................................................138 Polymyositis and dermatomyositis..............................................................................................244
Diabetes mellitus: overview and management..................................................................140 Psoriatic arthritis......................................................................................................................................246
Diabetic ketoacidosis...........................................................................................................................148 Reactive arthritis......................................................................................................................................248
Hyperaldosteronism.............................................................................................................................152 Rheumatoid arthritis.............................................................................................................................250
Hypocalcaemia........................................................................................................................................154 Scleroderma...............................................................................................................................................254
Hypercalcaemia and hyperparathyroidism............................................................................156 Septic arthritis...........................................................................................................................................256
Hyperprolactinaemia...........................................................................................................................158 Sjögren syndrome..................................................................................................................................258
Hypothyroidism.......................................................................................................................................160 Systemic lupus erythematosus......................................................................................................260
Hyperthyroidism.....................................................................................................................................164 Vitamin D deficiency............................................................................................................................264

vi / Contents Mind Maps for Medicine \ vii

 Chapter 8: Infectious diseases 267

Hepatitis A...................................................................................................................................................268
Hepatitis D and E....................................................................................................................................270
Hepatitis B...................................................................................................................................................272
Hepatitis C...................................................................................................................................................274
Human immunodeficiency virus..................................................................................................276
Malaria...........................................................................................................................................................280
Tuberculosis...............................................................................................................................................282
Preface

Appendix: Figure acknowledgements..................................................................................... 287 Mind maps (or similar concepts) have been used for mind map, taking readers consistently through
Index.............................................................................................................................................................. 293 centuries, for learning, brainstorming, visual thinking definition, pathophysiology, causes, clinical features,
and problem solving amongst educators, psychologists investigations, management, complications and more.
and people in general. They are particularly helpful The book is primarily aimed at medical students, but
for visual learners and help with memorisation and it should also be useful for junior doctors and general
retention, and are a more engaging form of learning, practitioners.
helping to improve productivity, efficiency and Chapters are largely laid out in alphabetical order
creativity. Studies on medical students have shown to allow for readers to easily locate medical conditions.
that using mind maps may yield better pass rates than There are a few exceptions, however, where it was felt
traditional note-taking. that some conditions should be grouped together in
It was during my second year of medical school terms of a spectrum from less to more severe, such
that I first really appreciated the value of mind maps as the sections covering angina pectoris and acute
after having a go at constructing some myself for the coronary syndrome, and also the sections on TIA
first time. It was certainly a game-changer for me. and stroke.
It enabled me to digest and retain a large amount For substantial topics where all the relevant and
of information in a structured manner, and therefore important information cannot feasibly fit onto one
resulted in a more efficient learning style. Prior to this mind map, additional supplementary information is
I had spent much more time making lengthy notes provided on a Notes page overleaf, in an easy to read
which I found far less productive and rather tedious. bullet point format.
It was at that point that the phrase ‘work smarter, not Additional memory aids in the form of mnemonics
harder’ hit home. During medical school, using mind are also included alongside photographs and
maps helped me with recall in written examinations, illustrations to further enhance retention and
but I also found that they enabled me to provide more visualisation.
structured and cogent answers in an OSCE setting, and It is important to note that this book is not intended
examiners seemed to prefer this to simply regurgitating as a replacement for larger textbooks but should be
lists of unstructured and undifferentiated information. seen as a companion to them, giving you a more
A few years ago I recognised the need for a medical innovative and fun way of learning.
revision guide set out in a mind map format – existing I hope that readers not only find this book enjoyable
books seemed to oversimplify their mind maps and not and useful in providing medical knowledge but that it
provide sufficient detail. They also failed to supplement also inspires you to create your own medical mind maps.
the written information with drawings and photos, Finally, I wish all readers the best with your medical
which I feel is vital in a visual memory aid. exams and future careers.
This book takes a systems-based approach and
for each of over 100 conditions presents a detailed Mohsin Azam

viii / Contents Mind Maps for Medicine \ ix

 Abbreviations
Acknowledgements
I would like to thank the student reviewers for their valuable feedback ABC Airway, breathing, circulation COPD Chronic obstructive pulmonary disease
on the content of some of the chapters. ABG Arterial blood gas CPAP Continuous positive airway pressure
ABPM Ambulatory blood pressure monitoring CRH Corticotropin-releasing hormone
I am extremely grateful to the team at Scion Publishing Ltd, particularly ACE Angiotensin-converting enzyme CRP C-reactive protein
Dr Jonathan Ray for his advice, encouragement and, above all, patience. ACR Albumin : creatinine ratio CSF Cerebrospinal fluid
ACS Acute coronary syndrome CT Computed tomography
ACTH Adrenocorticotropic hormone CTPA Computed tomography pulmonary angiogram
ADH Antidiuretic hormone CV Cardiovascular
ADL Activities of daily living CVA Cerebrovascular accident
Dedications AF Atrial fibrillation CVD Cardiovascular disease
AIDS Acquired immune deficiency syndrome CWP Coal worker’s pneumoconiosis
AKI Acute kidney injury CXR Chest X-ray
To my beloved parents for all the unconditional love and support
ALP Alkaline phosphatase DEXA Dual energy X-ray absorptiometry
they have given me throughout my life.
ALT Alanine transaminase DI Diabetes insipidus
To my sweetheart Sevda for the continued love and patience you have for me. AMA Anti-mitochondrial antibody DIDMOAD Diabetes insipidus, diabetes mellitus,
AMTS Abbreviated mental test score optic atrophy, and deafness
A special mention goes to my adorable baby daughter, Elanur, ANA Anti-nuclear antibody DIP Distal interphalangeal
you have made me a happy and proud father! ANCA Anti-neutrophil cytoplasmic antibody DKA Diabetic ketoacidosis
Anti-CCP Anti-cyclic citrullinated peptide DMARD Disease-modifying anti-rheumatic drug
APTT Activated partial thromboplastin time DOAC Direct oral anticoagulant
ARB Angiotensin II receptor blocker DVLA Driver and Vehicle Licensing Agency
ARDS Acute respiratory distress syndrome DVT Deep vein thrombosis
AS Ankylosing spondylitis EBV Epstein–Barr virus
ASMA Anti-smooth muscle antibodies ECG Electrocardiogram
AST Aspartate transaminase ECHO Echocardiogram
ATP Adenosine triphosphate EEG Electroencephalogram
AV Atrioventricular eGFR Estimated glomerular filtration rate
AVP Arginine vasopressin ELISA Enzyme-linked immunosorbent assay
AXR Abdominal X-ray EMG Electromyography
BE Base excess ERCP Endoscopic retrograde
BIPAP Bilevel positive airway pressure cholangiopancreatography
BNP Brain natriuretic peptide ESR Erythrocyte sedimentation rate
BP Blood pressure FBC Full blood count
CABG Coronary artery bypass grafting FEV Forced expiratory volume
CBT Cognitive behavioural therapy FSH Follicle-stimulating hormone
CCF Congestive cardiac failure FVC Forced vital capacity
CK Creatine kinase G6PD Glucose-6-phosphate dehydrogenase
CKD Chronic kidney disease GBS Guillain–Barré syndrome
CMV Cytomegalovirus GCA Giant cell arteritis
CNS Central nervous system GCS Glasgow Coma Scale
COCP Combined oral contraceptive pill GFR Glomerular filtration rate

x / Acknowledgements and Dedications Mind Maps for Medicine \ xi

 GI Gastrointestinal MI Myocardial infarction SDH Subdural haematoma TED Thrombo-embolus deterrent
GLP Glucagon-like peptide MND Motor neurone disease SHBG Sex hormone binding globulin TENS Transcutaneous electrical nerve stimulation
GORD Gastro-oesophageal reflux disease MPTP 1-methyl-4-phenyl-1,2,3,6-​tetrahydropyridine SIADH Syndrome of inappropriate ADH secretion TFTs Thyroid function tests
GRA Glucocorticoid-remediable aldosteronism MRA Magnetic resonance angiogram SLE Systemic lupus erythematosus TIA Transient ischaemic attack
GTN Glyceryl trinitrate MRCP Magnetic resonance cholangiopancreatography SMA Smooth muscle antibody TNM Tumour, node, metastasis
GU Genitourinary MRI Magnetic resonance image SNRI Serotonin and noradrenaline reuptake inhibitor TRH Thyrotropin-releasing hormone
HAART Highly active antiretroviral therapy MS Multiple sclerosis SNS Sympathetic nervous system TSH Thyroid-stimulating hormone
HAV Hepatitis A virus MSK Musculoskeletal SOB Shortness of breath TTG Tissue transglutaminase
HBA1c Haemoglobin A1c MSM Men who have sex with men SPECT Single photon emission computed tomography U&Es Urea and electrolytes
HBPM Home blood pressure monitoring MTP Metatarsophalangeal SS Sjögren syndrome UC Ulcerative colitis
HDU High dependency unit NAC N-acetyl cysteine SSRI Selective serotonin reuptake inhibitor UGIB Upper gastrointestinal bleeding
HDV Hepatitis D virus NBM Nil by mouth STEMI ST elevation myocardial infarction US Ultrasound
HELLP Haemolysis, elevated liver enzymes NG Nasogastric STI Sexually transmitted infection UTI Urinary tract infection
and low platelet count NICE National Institute for Health and Care Excellence T1DM Type 1 diabetes mellitus VF Ventricular fibrillation
HEV Hepatitis E virus NSAID Non-steroidal anti-inflammatory drug T2DM Type 2 diabetes mellitus VT Ventricular tachycardia
HF Heart failure NSCLC Non-small cell lung carcinoma TB Tuberculosis VTE Venous thromboembolism
HH Hereditary haemochromatosis NSTEMI Non-ST elevation myocardial infarction TCA Tricyclic antidepressant WCC White cell count
HHS Hyperosmolar hyperglycaemic state OA Osteoarthritis
HIV Human immunodeficiency virus OD Omnie die (once a day)
HLA Human leucocyte antigen OGTT Oral glucose tolerance test
HOCM Hypertrophic obstructive cardiomyopathy ON Omni nocte
HRT Hormone replacement therapy OSA Obstructive sleep apnoea
HSP Henoch–Schönlein purpura PBC Primary biliary cholangitis
HTN Hypertension PCI Percutaneous coronary intervention
IBD Inflammatory bowel disease PCOS Polycystic ovary syndrome
IBS Irritable bowel syndrome PD Parkinson’s disease
ICD Implantable cardioverter-defibrillator PE Pulmonary embolism
ICP Intracranial pressure PEF Peak expiratory flow
ICS Inhaled corticosteroid PEFR Peak expiratory flow rate
ICU Intensive care unit PENS Percutaneous electrical nerve stimulation
IDDM Insulin-dependent diabetes mellitus PET Positron emission tomography
IE Infective endocarditis PIP Proximal interphalangeal
IGF Insulin-like growth factor PM Polymyositis
IHD Ischaemic heart disease PMR Polymyalgia rheumatica
INR International normalised ratio PPI Proton pump inhibitor
IPF Idiopathic pulmonary fibrosis PSC Primary sclerosing cholangitis
ITU Intensive treatment unit PT Prothrombin time
IV Intravenous PTH Parathyroid hormone
JVP Jugular venous pressure PVD Peripheral vascular disease
KUB Kidneys, ureter, bladder RA Rheumatoid arthritis
LABA Long-acting beta agonist RAAS Renin–angiotensin–aldosterone system
LAD Left anterior descending RBBB Right bundle branch block
LBBB Left bundle branch block RCA Right coronary artery
LCx Left circumflex RF Rheumatoid factor
LDH Lactate dehydrogenase RR Respiratory rate
LFTs Liver function tests RV Right ventricular
LMWH Low molecular weight heparin SABA Short-acting beta agonist
LOS Lower oesophageal sphincter SAH Subarachnoid haemorrhage
LTRA Leukotriene receptor antagonist SALT Speech and language therapy
LVEF Left ventricular ejection fraction SAMA Short-acting muscarinic antagonist
LVH Left ventricular hypertrophy SBP Systolic blood pressure
MART Maintenance and reliever therapy SC Subcutaneous
MCP Metacarpophalangeal SCC Squamous cell carcinoma
MEN Multiple endocrine neoplasia SCLC Small cell lung carcinoma

xii / Abbreviations Mind Maps for Medicine \ xiii

 Chapter 1
01
Cardiology

Angina pectoris............................................................................................................................................................... 2
Acute coronary syndrome........................................................................................................................................ 4
Acute pericarditis............................................................................................................................................................ 8
Atrial fibrillation.............................................................................................................................................................10
Valvular heart disease................................................................................................................................................12
Heart failure......................................................................................................................................................................14
Hypertrophic obstructive cardiomyopathy.................................................................................................18
Hypertension..................................................................................................................................................................20
Infective endocarditis................................................................................................................................................24

Mind Maps for Medicine \ 1

 Non-modifiable risk factors Modifiable risk factors Symptoms • Variant (Prinzmetal’s) angina Caused by coronary artery spasm
(of normal coronary arteries on angiogram) and usually occurs at rest
• Increasing age • Smoking Classical presentation: without any provocation.
• Male sex • Diabetes mellitus (and impaired • Unstable angina Recurrent episodes of angina on minimal effort
Central crushing, retrosternal chest pain on exertion and relieved
• Family history of premature coronary glucose tolerance) or at rest (see Ch1: Acute coronary syndrome)
by rest within a few minutes. It may also be exacerbated by anger,
Angina refers to pain in the chest, heart disease • Hypertension • Cardiac syndrome X Describes patients with combination of angina-
excitement and cold weather, and frequently radiates to the arms and
neck, shoulders, jaw or arms caused • Premature menopause • Dyslipidaemia like chest pain, positive objective evidence of myocardial ischaemia
neck
by an insufficient blood supply • Obesity (e.g. positive exercise stress test) with a normal coronary angiogram
to the myocardium. • Sedentary lifestyle
Other variants: Signs
• Decubitus angina Occurs when the patient lies down
• Examination is usually normal but important to assess for risk factors
Definition Risk factors • Nocturnal angina Occurs at night and often wakes a patient from
sleep such as hypertension and signs of hyperlipidaemia (e.g. corneal arcus
and xanthelasma) and underlying causes e.g. aortic stenosis

Clinical features

Angina pectoris Notes

Angina pectoris

Conservative Management
Investigations
Mainly based on modifying risk factors:
• Smoking cessation
• Healthy diet ECG
• Exercise
• 12-lead ECG May show some ischaemic changes but a
• Weight loss
normal ECG does not rule out a diagnosis of angina
• Controlling diabetes, hypertension and raised cholesterol Revascularisation
• Exercise ECG Typically ST segment depression after
Pharmacological Coronary revascularisation is required in those at high risk and exercising usually diagnostic but normal test does not
(ABC Now Simply Note It Right away) those who fail to be controlled by medical therapy. The choice exclude diagnosis
of coronary artery bypass grafting (CABG) or percutaneous
• Aspirin All patients should receive aspirin 75mg OD for secondary Bloods
coronary intervention (PCI) depends on the distribution of the
prevention coronary artery disease, comorbidities and patient preference. FBC, U&Es, glucose/HbA1c, lipid profile, LFTs, TFTs, troponin
• Beta blocker e.g. Atenolol or Calcium channel blocker* depending on
comorbidities, contraindications and the person’s preference PCI ECHO
• Nitrates Sublingual glyceryl trinitrate (GTN) for acute symptomatic relief;
a long-acting nitrate can also be used 2nd line, e.g. isosorbide mononitrate Generally for patients with isolated coronary artery disease;
• Resting ECHO To assess cardiac function, or if hypertrophic
• Statin e.g. Atorvastatin in the absence of any contraindication localised atheromatous lesions are dilated using small
cardiomyopathy or aortic valve disease is suspected
• Nicorandil Dual properties of a nitrate and ATP-sensitive K+ channel agonist; inflatable balloons and then a stent is placed; complications
• Stress ECHO To assess for ischaemic regional wall
used as 2nd-line agent include death, acute MI, the need for urgent CABG and
changes while the patient is subjected to stress in the
• Ivabradine A selective inhibitor of sinus node pacemaker activity, restenosis.
form of exercise or chemically (if unable to exercise) with
used as 2nd-line agent dobutamine
• Ranolazine Reduces myocardial ischaemia by acting on intracellular CABG
Na+ currents, also used as 2nd-line agent The internal mammary artery or small saphenous vein is Coronary angiography
used to bypass the stenosis. It is particularly undertaken for
*If a calcium channel blocker is used as monotherapy then a rate-limiting one, • Gold standard for assessing coronary artery disease and
left main stem obstruction or triple vessel disease. The main
e.g. verapamil or diltiazem, should be used; if used in combination with a beta exact coronary artery anatomy before coronary intervention
complication is re-occurrence of angina due to accelerated
blocker then a long-acting dihydropyridine calcium channel blocker should be
atherosclerosis in the graft.
used, e.g. nifedipine; beta blockers should not be co-prescribed with verapamil Cardiovascular MR/CT angiography
(due to risk of complete heart block).
• Non-invasive investigation to assess coronary artery disease

2 / Chapter 1 Cardiology: Angina pectoris Mind Maps for Medicine \ 3

Acute coronary syndrome (ACS) encompasses a spectrum The underlying cause of coronary artery disease is atherosclerosis which has Non-modifiable • Chest pain Typically central and ‘crushing’ in nature and often radiates to
of unstable coronary artery diseases. It covers the several stages: arms, shoulders, neck or jaw
following diagnoses: • Endothelial cell injury and inflammation Lipids deposited in the • Increasing age • Shortness of breath
• Unstable angina Symptoms at rest with ECG changes; intima layer of coronary arteries together with vascular injury (e.g. from • Male sex • Nausea
the myocardial ischaemia is not sufficient to cause hypertension) cause inflammation, increased permeability and recruitment • Family history of premature coronary heart disease • Palpitations
myocardial damage and therefore there is no rise in of white cells; over time inflammatory cells, particularly macrophages, take • Premature menopause • Sweating
serum markers of myocardial injury e.g. troponin up the lipid and become foam cells
Note: Some patient groups, e.g. elderly, diabetics, females and those with CKD,
• Non-ST elevation myocardial infarction (NSTEMI) • Plaque formation Foam cells accumulate and smooth muscle cells Modifiable
may have little or no chest pain, and may present with atypical features such as
As above but with myocardial ischaemia sufficient to proliferate resulting in the growth of the plaque
• Smoking abdominal pain, altered mental state or jaw pain, so-called ‘silent MI’
cause myocardial damage and therefore elevation in • Plaque rupture Activates the clotting cascade and thrombosis which
serum markers of myocardial injury might be sufficient to cause partial occlusion (resulting in myocardial • Diabetes mellitus (and impaired glucose tolerance)
• ST elevation myocardial infarction (STEMI) ischaemia and angina) or total occlusion (resulting in myocardial necrosis • Hypertension
Symptoms with ST elevation on ECG due to complete and myocardial infarction, MI) • Dyslipidaemia Clinical features
occlusion of the coronary artery • Obesity
• Sedentary lifestyle
Pathophysiology
Definition Risk factors

Epidemiology • Coronary heart disease is the most

common cause of death in the UK
• Incidence increases with age, and
elderly people also tend to have
higher rates of morbidity and

Acute coronary syndrome

mortality from their infarcts

Investigations

ECG
Bloods
• Unstable angina and NSTEMI May be normal, show ST-segment
Complications Management depression or non-specific abnormalities e.g. T-wave inversion • Routine FBC (to exclude anaemia), U&Es (to assess
renal function), fasting lipids (risk stratification), LFTs
• STEMI Acute ST-segment elevation or new left bundle branch block (LBBB)
(see Figs. 1–3) (baseline before starting statins), TFTs
• Troponin and other cardiac enzymes Cardiac
Complications of ACS (DARTH VADER) Acute Normal Hours Days Weeks Months troponins T and I are highly sensitive and specific for
• Death/cardiac arrest Most commonly occurs due to MI (normal in unstable angina); other cardiac enzymes
patients developing ventricular fibrillation Initial* treatment for all patients (MONA) such as creatinine kinase will also be raised but this is
• Arrhythmias Tachyarrhythmias (e.g. VF and VT) or • Morphine Oral or IV diamorphine, e.g. 2.5–5mg, less specific
- ST elevation - ST elevation - ST flattening off - Pathological Q waves
bradyarrhythmias (atrioventricular block is more can be given for pain relief; an anti-emetic such as - Pathological Q waves - Pathological Q waves
metoclopramide should be co-prescribed - Inverted T waves CXR
common following inferior myocardial infarctions)
• Rupture Free ventricular wall/ventricular septum/ • Oxygen Should only be given if hypoxic, or evidence Fig. 1 ECG changes in MI
• To assess for presence or absence of heart failure/
papillary muscles of pulmonary oedema, or continuing myocardial
pulmonary oedema and exclude other diagnoses
• Tamponade and cardiogenic shock ischaemia
• Heart failure Acute or chronic • Nitrates To relieve ischaemic pain (initially sublingual, ECHO
• Ventricular Free wall or interVentricular septum rupture e.g. two sprays of GTN); if not effective, IV or buccal
• Aneurysm Of the left ventricle GTN or IV isosorbide dinitrate can be given • Can assess for regional wall abnormalities and
• Dressler syndrome Tends to occur around 2–6 weeks • Aspirin All patients should receive 300mg aspirin define the extent of the infarction and assess overall
following a MI (thought to be autoimmune) crushed or chewed ASAP ventricular function and can identify complications
• thromboEmbolism Mural thrombus
*Subsequent management depends on whether Coronary angiography
• Rupture Of the papillary muscle and mitral valve
the patient has unstable angina/NSTEMI or STEMI
Regurgitation
(see flow chart on Notes page overleaf) • Cardiac angiography defines the patient’s coronary
anatomy and the extent of the disease
Chronic

See Notes page overleaf.

Fig. 2 LBBB on ECG
4 / Chapter 1 Cardiology: Acute coronary syndrome Mind Maps for Medicine \ 5
 Acute coronary syndrome notes

ECG localisation of myocardial infarction Long-term treatment

NICE guideline (NG185) on the acute management of ACS
It is possible to localise the ischaemic area of the heart by assessing the leads Non-pharmacological
that show ST segment elevation (see Table 1). • Smoking cessation
Table 1 ECG localisation of myocardial infarction • Weight control
Initial treatment: • Diet Mediterranean-style diet is advised
• Morphine Leads Affected myocardial area Occluded coronary artery • Exercise Advise 20–30min/day until patients are ‘slightly breathless’; refer to
• Oxygen cardiac rehabilitation
• Nitrates V1-V2 Septal Proximal LAD • Driving If treated successfully by coronary angioplasty, group 1 driving
• Aspirin – 300mg orally (crushed or chewed) may recommence after 1 week; if not successfully treated, driving may
V3-V4 Anterior LAD recommence after 4 weeks
• Sexual activity May resume 4 weeks after uncomplicated MI; PDE5
V5-V6 Apical Distal LAD, LCx or RCA inhibitors (e.g. sildenafil) cannot be used until 6 months after MI and should
be avoided if patient is on nitrates or nicorandil
Unstable angina/NSTEMI *STEMI
1, AVL Lateral LCx
Pharmacological (DABS)
2, AVF, 3 Inferior 90% RCA, 10% LCx
• Dual antiplatelet therapy (DAPT) Aspirin 75mg OD lifelong and one other
Estimate 6m mortality, No antiplatelet (ticagrelor, prasugrel or clopidogrel) for at least 1 year; consider
Suitable for reperfusion therapy?
e.g. using GRACE score co-prescribing a PPI (e.g. omeprazole) for gastric protection
• Ace inhibitor or ARB (If intolerant to ACE inhibitor)
Yes • Beta blocker e.g. Bisoprolol, carvedilol and metoprolol; start low and
increase gradually as blood pressure and pulse allow; if contraindicated
consider calcium channel blocker e.g. verapamil or diltiazem
Low risk (≤3%) Intermediate/high risk (≥3%) PCI possible within 120min? • Statin High dose e.g. atorvastatin 80mg ON
• An aldosterone antagonist e.g. Eplerenone for patients who have had
an acute MI and evidence of heart failure and left ventricular systolic
Yes No dysfunction
Fig. 3 ECG showing anteroseptal MI
Typical contraindications to fibrinolytic therapy
Manage conservatively PCI Fibrinolysis Risk assessment tools for ACS
Table 2 Typical contraindications to fibrinolytic therapy
1. GRACE
• Give ticagrelor – initially For unstable angina/NSTEMI: For STEMI: Indicated if <12h of onset of Absolute Relative
• The Global registry of acute coronary events (GRACE) Score is a scoring
180mg for 1 dose, then • Immediate angiography if • Preferred strategy if <12h of symptoms if primary PCI cannot
system to risk stratify patients with diagnosed ACS to estimate their
90mg twice daily unstable, otherwise within onset of symptoms be delivered within 120min of the • Previous haemorrhagic stroke • Refractory hypertension
in-hospital and 6-month to 3-year mortality.
• Consider clopidogrel 300mg 72h if no contraindications • Give another antiplatelet – time when fibrinolysis could have • Ischaemic stroke in the previous (systolic BP >180mmHg)
• It is based on 8 variables: Age, signs of heart failure, heart rate and
loading dose – with aspirin, • Give another antiplatelet prasugrel prior to PCI been given 6 months • TIA in preceding 6 months
BP at presentation, serum creatinine level, ECG changes, troponin
or aspirin alone, for high prior to PCI – prasugrel (if not already taking • Options for fibrinolysis • Central nervous system trauma • Oral anticoagulant treatment
concentration and cardiac arrest at presentation
bleeding risk or ticagrelor (if not taking oral anticoagulant) or include alteplase, reteplase, or neoplasm • Pregnant or <1 week
2. TIMI
an oral anticoagulant) or clopidogrel (if taking an oral streptokinase or tenecteplase • Recent (<3 months) major postpartum
• The TIMI Risk Score for UA/NSTEMI estimates mortality for patients
clopidogrel (if taking an oral anticoagulant) • Give an antithrombin at the surgery, head injury or major • Traumatic cardiopulmonary
with unstable angina and NSTEMI.
anticoagulant) • Radial access is preferred and same time trauma resuscitation (CPR)
3. HEART score:
• Offer systemic unfractionated offer unfractionated heparin • Following procedure, give • Active internal bleeding or • Active peptic ulcer disease
• HEART Score for Major Cardiac Events. Predicts 6-week risk of major
heparin with bailout glycoprotein ticagrelor GI bleeding within last month • Advanced liver disease
adverse cardiac event. It is used in patients ≥21 years old presenting
2b/3a inhibitor • See Table 2 for typical • Known or suspected aortic • Infective endocarditis
with symptoms suggestive of ACS. HEART is an acronym and stands
• Drug-eluting stent should be contraindications to fibrinolysis dissection • Previous allergy to fibrinolytic
for 5 variables: History, ECG, Age, Risk factors, and Troponin.
used in preference • Known bleeding disorder drugs
*Clinical symptoms consistent with ACS with persistent ECG features in ≥2 contiguous leads of:
• 2.5mm ST elevation in leads V2–3 in men <40y, or ≥2.0mm ST elevation in leads V2–3 in men ≥40y
• 1.5mm ST elevation in V2–3 in women
• 1mm ST elevation in other leads
• new LBBB (LBBB should be considered new unless there is evidence otherwise)

6 / Chapter 1 Cardiology: Acute coronary syndrome notes Mind Maps for Medicine \ 7
 • Viral infection (e.g. coxsackievirus, mumps, EBV, Symptoms Pericardial effusion and cardiac Constrictive pericarditis
CMV, varicella, HIV)
• Tuberculosis • Chest pain: may be pleuritic and often
tamponade Definition
• Uraemia (e.g. in severe renal failure) relieved by sitting forwards Definition Constrictive pericarditis is caused by a rigid pericardial sac that limits
• Trauma • Non-specific symptoms: non-
• Post myocardial infarction, including Dressler productive cough, dyspnoea ventricular filling.
Pericardial effusion describes a collection of fluid in the pericardial space
syndrome and flu-like symptoms which may result from any of the causes of pericarditis. It can lead to cardiac
• Connective tissue disease e.g. SLE, RA, polyarteritis Causes
tamponade which is a form of cardiogenic shock from restricted diastolic
nodosa Signs ventricular filling caused by a large amount of fluid accumulation in the • Tuberculosis
• Rheumatic fever pericardial space. It is a medical emergency.
• Pericardial friction rub • Viral infection
• Hypothyroidism
• Tachypnoea • Malignancy: carcinomatous invasion of the pericardium
• Malignancy Clinical features
Acute pericarditis is inflammation of • Tachycardia • Radiotherapy of the chest
• Iatrogenic: radiotherapy, post cardiac surgery,
the pericardium. • Renal failure
drugs (e.g. procainamide, hydralazine) • Fatigue
• Post cardiac surgery
• Breathlessness characteristically, occurs on exertion
• Ascites
Definition Causes Clinical features • Peripheral oedema
Clinical features
• Pulse: pulsus paradoxus, AF, tachycardia • Fatigue
• Kussmaul’s sign (a raised JVP with inspiration) – rare • Breathlessness characteristically, occurs on exertion
• Cardiac impulses: barely palpable; characteristic is systolic retraction • Ascites
at the apex • Peripheral oedema
• Pericardial knock (loud-high pitched S3) following S2 • Pulse: AF, tachycardia
• Beck’s triad is the hallmark of cardiac tamponade (see Fig. 2) • Kussmaul’s sign (a raised JVP with inspiration)
• JVP – an absent Y descent • Cardiac impulses: barely palpable; characteristic is systolic retraction
at the apex
• Pericardial knock (loud-high pitched S3) following S2
Acute pericarditis ↑JVP Hypotension Investigations

Beck’s triad • ECG: may show low-voltage QRS complexes and generalised T-wave
inversion
• CXR: small cardiac shadow; there may be peripheral calcification
• ECHO: thickened pericardium; a normally contracting ventricle
Complications Muffled heart sounds
(unlike the ventricle seen in a restrictive cardiomyopathy)
Fig. 2 Beck’s triad
Investigations Management
• Pericardial effusion and cardiac Investigations
tamponade • Pericardiectomy (surgical excision of the pericardium)
• Chronic pericarditis • CXR: large globular heart
• Constrictive pericarditis • ECG: may show ST elevation with MI or pericarditis, or loss of voltages;
alternating QRS morphologies
• ECHO: diagnostic (echo-free zone surrounds the heart)
ECG Bloods
• Cardiac MRI or CT: may be superior to ECHO in detecting loculated
• Widespread ‘saddle-shaped’ ST elevation (see Fig. 1) • FBC, U&Es, CRP/ESR, troponin pericardial effusions and pericardial thickening
• Sinus tachycardia is common (may be raised), viral • Diagnostic pericardiocentesis: pericardial fluid sent for microbiological and
• T waves may initially be prominent, upright and peaked serology, autoantibodies cytological testing
• AF, atrial flutter or atrial topics may occur (e.g. ANA, RF, anti-CCP if
Management
Management • PR depression: most specific ECG marker for pericarditis indicated) and TFTs to assess
for underlying causes if • Urgent therapeutic pericardiocentesis is required
indicated
• Treat the underlying cause
• A combination of NSAIDs and CXR
colchicine is 1st line for patients
with acute idiopathic or suspected • May show globular
viral pericarditis enlargement of the heart
if there is presence of
pericardial effusion

ECHO

• Confirms the presence of a

Fig. 1 ECG showing widespread saddle-shaped ST elevation in a patient with acute pericardial effusion
pericarditis

8 / Chapter 1 Cardiology: Acute pericarditis Mind Maps for Medicine \ 9

 Cardiac Non-cardiac Symptoms Signs

• Ischaemic heart disease • Sepsis • Palpitations • Irregularly irregular

Atrial fibrillation (AF) is the most common • Results from irregular, disorganised electrical activity in the atria • Heart failure • PE • Dyspnoea pulse
tachyarrhythmia. It is characterised by an irregular, from rapidly firing cells at the junction of the pulmonary veins in • Hypertension • Thyrotoxicosis • Chest pain • Signs of heart failure
disorganised electrical activity in the atria. AF can be the left atrial musculature • Valvular heart disease • Lung or pleural disease • Dizziness
grouped into: • The rapidly firing impulses cause disorganised atrial depolarisation • Sick sinus syndrome • Chest trauma • Syncope
• Paroxysmal AF Episodes lasting >30sec but <7 days and ineffective atrial contractions • Pericarditis • Hypokalaemia • Fatigue
that are self-terminating and recurrent • The atrioventricular (AV) node receives more electrical impulses • Infiltrative heart disease • Hypovolaemia
• Persistent AF Episodes lasting >7 days than it can conduct, resulting in an irregular ventricular rhythm • Cardiomyopathy • Hypothermia
• Permanent AF AF that fails to terminate using • This may result in stagnant blood accumulating within the atrial • Myocarditis • Alcohol abuse
cardioversion, AF that is terminated but relapses within appendage, ↑risk of clot formation and hence embolic stroke • Congenital heart disease • Drug abuse e.g. cocaine
24h, or long-standing AF (usually longer than 1 year)
Clinical features

Definition Pathophysiology
Causes

Atrial fibrillation Complications

• Stroke (5-fold risk)

• Heart failure
• Tachycardia-induced cardiomyopathy
and critical cardiac ischaemia

Management
Investigations
Rate control
Anticoagulation
BCD (better option in >65 years or hx of ischaemic heart
disease): In order to reduce the risk of stroke: ECG CXR
• Beta blockers e.g. bisoprolol, carvedilol, nebivolol • The CHA2DS2-VASc score helps determine the need for anticoagulation (see Box 1)
• Calcium channel blockers (rate limiting) • The risk of using anticoagulants can be calculated using the HASBLED score • Lack of P waves, irregularly irregular rhythm (see Fig. 1) • May indicate cardiac structural causes of AF, such as
e.g. diltiazem, verapamil (see Box 2) mitral valve disease, or heart failure
• Digoxin is preferred if there is co-existing heart • Patients should be offered a choice of anticoagulation from warfarin and the novel
failure or hypotension oral anticoagulants (NOACs) e.g. rivaroxaban, apixaban or dabigatran ECHO

Rhythm control • For whom a rhythm-control strategy that includes

Box 1 CHA2DS2-VASc score Box 2 HASBLED score
cardioversion is being considered, in whom there is
This is better option for <65 years, symptomatic, 1st a high risk or a suspicion of underlying structural/
presentation, lone AF or AF 2° to a precipitant or CCF: Fig. 1 ECG showing AF (note lack of P waves and irregularly irregular rhythm)
Congestive cardiac failure = 1 HTN (systolic BP >160mmHg = 1 functional heart disease
• Chemical:
– Sotalol HTN (or treated) = 1 Abnormal renal function (=1) or liver function (=1)
Holter monitoring CT or MRI brain
– Amiodarone (in the presence or absence Age ≥75 = 2, age 65–74 = 1 Stroke, history of = 1
of structural heart disease) Diabetes = 1 Bleeding, hx of bleeding or high risk = 1 • Should be performed if there is any suggestion of
S2 Prior stroke or TIA = 2 Labile INRs (<60% in therapeutic range) = 1 • 24–72h ECG for people with suspected paroxysmal AF undetected
– Flecainide (in the absence of structural heart by standard ECG recordings stroke or TIA
disease) Vascular disease Elderly (>65 years) = 1
• Electrical: (including IHD or PVD) = 1 Drugs predisposing to bleeding (=1)
Bloods
– Can be used in the acute scenario if the patient Sex (female) = 1 or alcohol use (=1)
is haemodynamically unstable (≥2 offer anticoagulation) (≥3 indicates a ‘high risk’ of bleeding) • FBC, U&Es, TFTs, magnesium, LFTs and coagulation screen
– Also can be done as an elective procedure
where a rhythm control strategy is preferred

10 / Chapter 1 Cardiology: Atrial fibrillation Mind Maps for Medicine \ 11

 Valvular heart disease

Aortic stenosis Aortic regurgitation (AR) Mitral stenosis Mitral regurgitation (MR)
Causes Causes Causes Causes

• Degenerative calcification (most common cause in patients >65 years) Due to aortic root disease • Rheumatic heart disease • Rheumatic heart disease
• Congenital bicuspid valve (most common cause in patients <65 years) • Aortic dissection • Calcification of valve • Papillary muscle rupture or rupture of chordae tendineae
• Post-rheumatic disease • Hypertension • Rheumatoid arthritis • Infective endocarditis
• Williams syndrome (supravalvular aortic stenosis) • Spondyloarthropathies e.g. ankylosing spondylitis • Ankylosing spondylitis • Mitral valve prolapse (common condition occurring mainly in young
• Hypertrophic obstructive cardiomyopathy (HOCM): subvalvular • Syphilis • SLE women)
• Marfan syndrome, Ehlers–Danlos syndrome • Malignant carcinoid • Hypertrophic cardiomyopathy
Clinical features • Ehlers–Danlos syndrome
Clinical features
Symptoms Signs Due to valve disease Clinical features
• Syncope • Narrow pulse pressure • Rheumatic fever Symptoms Signs
• Dyspnoea • Slow rising pulse • Infective endocarditis • Dyspnoea • Malar flush Symptoms Signs
• Angina • Thrill • Connective tissue diseases e.g. RA/SLE • Palpitations (if in AF) • Tapping apex beat • Dyspnoea • Irregularly irregular pulse
• Ejection systolic murmur radiating • Bicuspid aortic valve • Heart failure • Hoarse voice (Ortner syndrome) • Palpitations (if AF present) (if AF present)
to carotids • Haemoptysis • Irregularly irregular pulse • Heart failure symptoms • Displaced apex beat
• Soft/absent S2 Clinical features • Loud S1, opening snap • Harsh pansystolic murmur
• S4 • Mid–late diastolic murmur radiating to the axilla
Symptoms: Signs: (best heard in expiration) • Soft S1, split S2
Investigations • Dyspnoea • Waterhammer/collapsing pulse
• Angina • Wide pulse pressure Investigations Investigations
• ECG May show evidence of left ventricular • Heart failure • Early diastolic murmur
hypertrophy (see Fig. 1) or left ventricular • Mid-diastolic Austin Flint murmur • ECG May show AF or P mitrale • ECG AF, P mitrale (bifid P waves)
strain (in severe AR) (bifid P waves) (see Fig. 3) • CXR May see cardiomegaly, and pulmonary oedema (if HF present)
• CXR May show post-stenotic dilatation • Traube’s sign: ‘pistol shot’ heard • CXR May show pulmonary • ECHO Confirms diagnosis and assesses severity
of ascending aorta (see Fig. 2) and Fig. 1 Left ventricular hypertrophy oedema and enlarged left
over the femoral artery
calcification of valve on ECG
• De Musset’s sign: head nodding in atrium Management
• ECHO Gold standard for diagnosis time with each heart beat • ECHO Confirms diagnosis and
• Multi-slice CT (MSCT) and cardiac MRI assesses severity • In acute cases, medical management with nitrates, diuretics, positive
• Quincke’s sign: nail bed pulsation
May be useful in providing additional Fig. 3 ECG showing P mitrale inotropes and an intra-aortic balloon pump can be used to increase cardiac
information to above prior to surgery Investigations Treatment output
• Cardiac catheterisation To measure • If patients are in heart failure ACE inhibitors, beta blockers and
pressures across the valve to assess the • CXR May show cardiomegaly, pulmonary oedema and dilatation of the • Asymptomatic patients can be managed conservatively by following the spironolactone should be considered
severity of disease and the need for ascending aorta patient clinically and with serial ECHO • The evidence for repair over replacement is strong in degenerative
intervention • ECG May show evidence of left ventricular hypertrophy • Diuretics or long-acting nitrates can be used to alleviate dyspnoea; beta regurgitation due to lower mortality and higher survival rates
• Coronary angiography May be indicated • ECHO Confirms diagnosis and assesses severity blockers or heart rate regulating calcium-channel blockers, e.g. verapamil, • When this is not possible, valve replacement with either an artificial valve or
as part of the assessment of coronary Fig. 2 Post-stenotic dilatation of • MSCT and cardiac MRI May be required for further evaluation can improve exercise tolerance a pig valve is considered
artery disease ascending aorta • Cardiac catheterisation May be used to assess coronary anatomy before • Anticoagulant therapy is indicated in patients with either permanent or • In acute severe regurgitation, emergency valve replacement is necessary
surgery paroxysmal AF
Management • Percutaneous mitral commissurotomy (PMC) should be considered for Complications
Management symptomatic patients with severe mitral stenosis or those with pulmonary
• If asymptomatic and the patient has mild to moderate aortic stenosis then hypertension • AF
the patient should be observed as a general rule • Patients with mild-to-moderate AR can be reviewed on a yearly basis and • Surgical valve replacement should be considered for patients who are not • Heart failure
• If symptomatic then patients should have valve replacement; if echocardiography performed every 2 years candidates for percutaneous intervention • Infective endocarditis
asymptomatic but severe with valvular gradient >40mmHg and with • Treat heart failure with diuretics, ACE inhibitors/ARBs and beta blockers • Pulmonary hypertension
features such as left ventricular systolic dysfunction then surgery • Surgery is usually indicated in symptomatic patients or asymptomatic Complications
should be considered patients when left ventricular function begins to deteriorate
• Balloon valvuloplasty is limited to patients with critical aortic stenosis who • Valve replacement remains the most widely used technique • AF
are not fit for valve replacement • Valve-sparing aortic root replacement is increasingly employed in expert • Heart failure
• TAVI (transcatheter aortic valve implantation) is a relatively new procedure centres, particularly in young patients • Infective endocarditis
which is less invasive, making it a consideration in patients who are
unsuitable for surgical aortic valve replacement Complications

Complications • Heart failure

• Arrhythmia
• Heart failure • Systemic emboli • Infective endocarditis
• Infective endocarditis • Sudden death
12 / Chapter 1 Cardiology: Valvular heart disease Mind Maps for Medicine \ 13
Heart failure (HF) is the inability Systolic vs diastolic HF Acute HF vs chronic HF • Myocardial disease: coronary artery • HF results from injury to the myocardium from a
Na+/H2O retention
of the heart to provide adequate disease (most common), hypertension, variety of causes (see Causes)
circulation at normal filling • Systolic HF or ‘HF with impaired ejection fraction’ • Acute HF Results from sudden failure to maintain cardiomyopathies • As the heart fails, there are several compensatory Vasoconstriction
pressures to meet the body’s Inability of the ventricles to contract properly resulting cardiac output; there is insufficient time for • Valvular heart disease: e.g. aortic stenosis mechanisms that occur as the failing heart
metabolic demands. It is caused in ↓cardiac output; ejection fraction <40%; causes compensatory mechanisms to develop • Arrhythmias: e.g. AF and other arrhythmias attempts to maintain adequate function ↑Afterload ↑Preload

by structural or functional include ischaemic heart disease and cardiomyopathy • Chronic HF Cardiac output declines gradually; • Pericardial disease: pericardial effusion, • These include increasing cardiac output via
abnormalities of the heart. • Diastolic HF or ‘HF with preserved ejection symptoms related to compensatory mechanisms constrictive pericarditis and cardiac the Frank–Starling mechanism, increasing Heart rate/contractility
↑Cardiac workload

fraction’ Inability of the ventricles to relax therefore predominating tamponade ventricular volume and eventually increased
Direct cardiotoxicity
fill adequately; ejection fraction >40%; causes include • Congenital heart disease: e.g. atrial wall thickness through ventricular remodelling, Myocyte damage

Definition cardiac tamponade, constrictive pericarditis and Low-output HF vs high-output HF septal defect (ASD) and ventricular and maintaining tissue perfusion with increased
restrictive cardiomyopathy septal defect (VSD) mean arterial pressure through activation of ↓Cardiac input
• Low-output HF Cardiac output is decreased and • High-output states: anaemia, thyrotoxicosis, sympathetic nervous system (SNS) and the
Left HF vs right HF unable to meet the demands of the body liver failure, Paget’s disease, beriberi renin–angiotensin–aldosterone system (RAAS) SNS activation RAAS activation

• High-output HF Rare; output is normal or • Drugs: alcohol, steroids, chemotherapy, • Although initially beneficial in the early stages Fig. 1 Pathophysiology of heart failure outlined
• Left ventricular failure or right ventricular failure may increased in the face of increased needs e.g. NSAIDs of HF, all of these compensatory mechanisms
occur independently or together as congestive cardiac Paget’s disease, hyperthyroidism, anaemia • Severe lung disease: e.g. COPD, obstructive eventually lead to a vicious cycle of worsening
failure (CCF) sleep apnoea, pulmonary embolism HF (see Fig. 1).
Pathophysiology
Classification Causes
Prognosis Signs

• Prognosis is poor on the whole, with Left heart failure

approximately 50% of people with HF Clinical features • Displaced apex beat
dying within 4 years of diagnosis • Gallop rhythm (3rd heart sound)

Heart failure
• The mortality rate in the UK appears • Inspiratory crackles/wheeze
to be improving and this is mainly Symptoms • Pitting oedema (see Fig. 2)
down to pharmacological agents and Right heart failure
• Dyspnoea
designated HF multidisciplinary teams • Raised jugular venous pressure (JVP) (see Fig. 3)
• Fatigue
• Oedema • Hepatic enlargement
• Diet and fluid intake Cachectic patients
• Beta blockers Staging severity • Nocturnal cough ± pink frothy • Ascites
Management should be assessed by a dietitian; restrict dietary
• Spironolactone sputum or wheeze • Pitting oedema
salt; patients with severe CCF should restrict • Orthopnoea
• Hydralazine with nitrates The New York Heart Association (NYHA) Classification
their fluid intake; encourage weight monitoring • Paroxysmal nocturnal
Acute heart failure • Alcohol Restrict alcohol intake or advise Drugs for symptomatic relief only: of HF is used to stage HF according to symptoms:
dyspnoea (PND)
abstention • Loop or thiazide diuretics • Class I No limitations; ordinary physical activity does
• Nocturia, cold peripheries, External
1. Sit patient up • Exercise Aerobic exercise, preferably a • Digoxin not cause symptoms
weight loss and muscle wasting Jugular Vein
2. High-flow oxygen supervised cardiac rehabilitation programme • Ivabradine • Class II Symptomatically ‘mild’; slight limitation of
3. IV furosemide e.g. 40mg IV (further boluses • Travel NYHA class I and II are not restricted physical activity but comfortable at rest Path of IJ
or IV infusion may be given) in plane travel; O2 may be required for class III • Class III Symptomatically ‘moderate’; marked limitation Clavicular
Surgical: of physical activity Head of
4. Diamorphine e.g. 2.5–5mg IV (if patient has and is recommended (with in-flight medical SCM
chest pain or is distressed and is not confused • Revascularisation Coronary artery disease • Class IV Symptomatically ‘severe’; symptoms of HF are
assistance) for class IV
or drowsy) with an anti-emetic is the most common cause of HF and if this is present even at rest Clavicle
• Sexual health No specific restrictions for sexual
5. If BP stable (i.e. >100 systolic) consider GTN the cause, revascularisation with angioplasty
activity but slight risk of decompensation with Sternal Head of SCM
spray e.g. 2 puffs sublingual or an infusion and stenting or coronary artery bypass surgery
NYHA class III–IV patients; sexual dysfunction
6. Consider catheterisation to monitor urine (CABG) can result in improvement Fig. 3 Jugular venous distension; SCM,
is common in HF patients due to the condition
output carefully itself or from the side effects of treatment
• Cardiac resynchronisation therapy Also Investigations Fig. 2 Pitting oedema of the lower limbs
sternocleidomastoid muscle; IJ, internal jugular
7. Treat underlying causes e.g. MI or arrhythmias known as biventricular pacing; it aims to
• Mental health and wellbeing Depression
8. Consider CPAP improve the coordination of the atria and
is very common in HF; screen and manage • Bloods FBC, U&Es, LFTs, TFTs, lipid profile, BNP (B-type natriuretic
9. If BP low (i.e. <100 systolic) consider ITU ventricles; it is indicated for patients with systolic
accordingly peptide) or N-terminal pro-BNP (NT-proBNP)
admission and inotropes, e.g. IV dobutamine HF who have moderate–severe symptoms and
• Immunisation Annual influenza vaccination • Urinalysis Nephrotic syndrome may present with fluid overload Prominent upper
for treatment of cardiogenic shock a widened QRS complex on ECG
and single pneumococcal vaccination should • CXR (ABCD) Alveolar oedema, Kerley B lines, Cardiomegaly, Dilated lobe vessels
• Implantable cardioverter-defibrillator (ICD)
be given upper lobe vessels, pleural Effusion (see Fig. 4) Alveolar oedema
Chronic heart failure For patients at high risk of lethal arrhythmias
• ECG May indicate cause e.g. MI, AF or show LVH (’Bat’s wings’)
such as ventricular tachycardia
Pharmacological: • ECHO May indicate the cause (MI, valvular heart disease) and confirm the
Conservative: • Cardiac transplantation The treatment of Kerley B lines
presence or absence of systolic or diastolic function and assess severity Cardiomegaly
• Stop any offending drugs if possible, such See notes on the next page Pharmacological agents choice for younger patients with intractable HF (interstitial oedema)
• Lung function tests To rule out respiratory disease
as NSAIDs, some calcium channel blockers, for heart failure. • Left ventricular assist device and artificial Pleural effusion
• Cardiac MRI The gold standard for assessing ventricular volumes, mass
e.g. verapamil heart For severe HF not controlled with above
Drugs that reduce mortality: and wall motion; can also be used to identify inflammation, infiltration
• Smoking cessation measures; it is occasionally given to people on
• ACE inhibitors/angiotensin receptor blockers and scarring of the myocardium Fig. 4 Signs of heart failure seen on CXR
the waiting list for a heart transplant
(ARBs)
14 / Chapter 1 Cardiology: Heart failure Mind Maps for Medicine \ 15
 Heart failure notes
Pharmacological agents for heart failure

Table 1 Cut off-values for BNP and NT-proBNP ACE inhibitors • Examples include ramipril, lisinopril and enalapril
Natriuretic peptides
• All patients with left ventricular ejection fraction (LVEF) ≤40%, regardless of symptom severity, should receive
BNP NT-proBNP
• B-type natriuretic peptide (BNP) is released into the blood when an ACE inhibitor unless contraindicated or not tolerated
the ventricular myocardium is stressed • ACE inhibitors improve ventricular function and patient wellbeing and reduce mortality
High level >400pg/ml >2000pg/ml • Start at low dose and titrate upwards at short intervals (e.g. every 2 weeks) until target or maximum tolerated
• The physiological action of BNP is to reduce systemic vascular
resistance and central venous pressure as well as an increase dose is reached
Raised level 100–400pg/ml 400–2000pg/ml • Common side effects include a dry cough (around 15% of patients and may occur up to a year after starting
in natriuresis
• The net effect is a decrease in blood pressure and a decrease treatment), angioedema, hyperkalaemia and hypotension
Normal level <100pg/ml <400pg/ml
in preload and afterload of the heart
• The N-terminal prohormone of brain natriuretic peptide (NT-proBNP)
is a prohormone with an N-terminal inactive protein that is cleaved Table 2 Factors that increase and decrease BNP/NT-proBNP Angiotensin receptor • Candesartan and valsartan are examples of ARBs that are licensed for HF
from the molecule to release BNP blockers (ARBs) • Indicated for patients who cannot tolerate an ACE inhibitor because of side effects; they do not cause the
Increase BNP levels Decrease BNP levels chronic cough side effect associated with ACE inhibitors
• Raised levels of BNP and NT-proBNP are suggestive of heart failure
(see Table 1) and very high levels are associated with a poor prognosis
• BNP may also be lowered and raised by other factors and it is Left ventricular hypertrophy Obesity
Ischaemia Diuretics Beta blockers • Examples of beta blockers licensed for heart failure include bisoprolol, nebivolol and carvedilol
important to take these into account when interpreting the result
Tachycardia ACE inhibitors • Beta blockers should be used in all patients with symptomatic HF and LVEF ≤40%, where tolerated and not
(see Table 2)
Right ventricular overload Beta blockers contraindicated
Hypoxaemia (including PE) Angiotensin 2 receptor blockers • Common side effects include: bronchospasm, fatigue, cold peripheries and sleep disturbances
GFR <60ml/min Aldosterone antagonists • Asthma, 2nd- or 3rd-degree heart block, sick sinus syndrome (without a pacemaker) and sinus bradycardia
Sepsis (<50bpm) are contraindications to beta-blocker use
COPD
Diabetes
Age >70 years Mineralocorticoid • Include spironolactone and eplerenone
Liver cirrhosis (aldosterone) receptor • Relatively weak diuretics with a K+-sparing action
antagonists • Spironolactone e.g. 25mg reduces mortality in patients with HF with impaired ejection fraction
• Epleronone reduces mortality in patients with acute MI and HF
• Gynaecomastia is a common side effect
• Main contraindication is hyperkalaemia

Management of chronic heart failure: a stepwise approach Vasodilators • Nitrates e.g. isosorbide mononitrate (reduces preload) in combination with hydralazine (reduces afterload)
improve symptoms and survival in patients with systolic HF
• Should be considered in patients who are intolerant to ACE inhibitors or ARBs

1. ACE inhibitors and beta blockers Use ARBs if patient intolerant to ACE inhibitors,
e.g. valsartan 80mg OD Digoxin • Considered as an add-on treatment in patients who remain symptomatic despite above treatments
e.g. ramipril 1.25mg OD, bisoprolol 1.25mg OD
• Useful in patients who have a combination of AF and HF

Ivabradine • Should be considered in patients that remain symptomatic despite already on suitable therapy (ACE inhibitor,
beta blocker + aldosterone antagonist), and have a heart rate >75/min and a left ventricular fraction <35%
2. Mineralocorticoid receptor antagonist Consider hydralazine with nitrates, e.g. isosorbide
e.g. spironolactone 25mg OD or eplerenone 25mg OD mononitrate if intolerant to ACE inhibitors or ARBs Sacubitril / valsartan • Contains sacubitril and valsartan (ARB)
(Entresto®) • Sacubitril (a prodrug) inhibits the breakdown of natriuretic peptides resulting in varied effects including
increased diuresis, natriuresis and vasodilatation
• Indications include symptomatic chronic HF with ejection fraction <35% and patients not currently taking an
ACE inhibitor or ARB, or stabilised on low doses of either of these agents

3. Digoxin, hydralazine with nitrates, cardiac resynchronisation

Diuretics • Includes loop diuretics e.g. furosemide 40mg (oral, IV bolus or infusion) and bumetanide 1mg (oral)
therapy or ivabradine or sacubitril with valsartan
• Thiazide diuretics e.g. bendroflumethiazide or indapamide are mild diuretics; the exception is metolazone
which causes profound diuresis and is only used for severe and resistant cases by specialists
• Spironolactone and eplerenone are relatively weak diuretics with K+-sparing action

16 / Chapter 1 Cardiology: Heart failure notes Mind Maps for Medicine \ 17

 • The most common defects involve a mutation • Often asymptomatic (and detected through family Dilated cardiomyopathy • Infiltrative myocardial disease e.g. iron in haemochromatosis, glycogen in
in the gene encoding beta-myosin heavy chain screening) Pompe and Cori disease, or glycolipids in Fabry disease
protein or myosin-binding protein C • Exertional dyspnoea Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy • Amyloid heart disease is the most common cause of restrictive
• It is characterised by myofibrillar hypertrophy with • Angina (accounting for 90% of cases). It is characterised by a dilated left ventricle cardiomyopathy in the West
Hypertrophic obstructive chaotic and disorganised myocytes (‘disarray’) and • Syncope (typically following exercise) which contracts poorly. • Sarcoidosis
cardiomyopathy (HOCM) is an fibrosis on biopsy • Sudden death, arrhythmias, heart failure
autosomal dominant disorder • This results in: • Jerky pulse, large ‘a’ waves, double apex beat Causes Clinical features
of muscle tissue leading to left • Left ventricular hypertrophy (LVH) → decreased • Ejection systolic murmur: increases with Valsalva
• Idiopathic: the most common cause • Features of right ventricular failure predominate
ventricular hypertrophy. It is the compliance → impairs diastolic filling → manoeuvre and decreases on squatting
• Myocarditis: e.g. Coxsackie B, HIV, diphtheria, Chagas’ disease • Clinical features resemble those of constrictive pericarditis
most common genetic heart disease decreased cardiac output • Pansystolic murmur if mitral regurgitation is
• Ischaemic heart disease (see Ch1: Acute pericarditis: constrictive pericarditis)
and the main cause of sudden • Sudden death is most commonly due to present (hypertrophic cardiomyopathy may impair
• Peripartum • AF is common (see Ch1: Atrial fibrillation)
cardiac death in the young. ventricular arrhythmias mitral valve closure)
• Hypertension
• Drugs: iatrogenic (e.g. doxorubicin), substance misuse (e.g. alcohol and Investigations
cocaine)
Definition Pathophysiology Clinical features • Inherited: the majority of defects are inherited in an autosomal dominant • Initial investigations as for heart failure: ECG, CXR, blood tests
(see Ch1: Heart failure)
fashion although other patterns of inheritance are seen
• Infiltrative: e.g. haemochromatosis, sarcoidosis • Echocardiography usually shows thickened ventricular walls, valves and
• Nutritional: e.g. wet beriberi (thiamine deficiency) atrial septum with small cavities
• Cardiac MRI is useful at distinguishing restrictive cardiomyopathy from
Clinical features constrictive pericarditis

• Classic findings of heart failure Management

• Systolic murmur: from mitral and tricuspid regurgitation
• S3 • Prognosis is poor and there is no specific treatment
• Heart failure and AF are treated in the usual way
Investigations • Amiodarone can reduce ventricular arrhythmias in high-risk patients
Hypertrophic obstructive • ICD to prevent sudden death in high-risk patients

cardiomyopathy
• CXR: cardiomegaly, pulmonary oedema • Transplantation may be indicated for some patients
• BNP: may be raised (especially in heart failure) and useful for prognosis
• ECG: may show sinus tachycardia, an intraventricular conduction delay, LBBB
or non-specific changes in ST and T waves Arrhythmogenic right ventricular
• ECHO: marked dilatation of the left ventricular cavity and reduced systolic cardiomyopathy
and diastolic function; may also show mitral regurgitation, tricuspid
regurgitation and mural thrombus Definition
• Cardiac MRI: dilated ventricles with global hypokinesis
Management Investigations • Endomyocardial biopsies may diagnose infiltrative disease Arrhythmogenic right ventricular cardiomyopathy (ARVC) is caused
by fibro-fatty replacement of right ventricular (RV) myocytes due to apoptosis,
Management inflammation (due to unknown cause) or a genetic cause.
Medical ECG
• Heart failure and AF treated in conventional way (see Ch1: Atrial fibrillation Clinical presentation and diagnosis
• Amiodarone suppresses atrial and ventricular arrhythmias • LVH, non-specific ST segment and T-wave and Heart failure)
• Beta blockers, verapamil and disopyramide reduce left abnormalities and progressive T-wave inversion may • Biventricular pacing (using a cardiac resynchronisation device) can improve • It usually presents with symptomatic arrhythmias e.g. ventricular tachycardia
ventricular outflow tract gradient and diastolic dysfunction be seen; deep Q waves and atrial fibrillation (AF) may symptoms in patients with class III and IV heart failure with marked QRS or sudden death
• Anticoagulation may be required for AF occasionally be seen prolongation, improve survival and increase exercise tolerance • Diagnosing ARVC is challenging due to non-specific disease features and
(see Ch1: Atrial fibrillation) • ICD reduces risk of sudden death in high-risk patients phenotypic manifestations
ECHO (MR SAM ASH) • Mitral annuloplasty or valve replacement can improve symptoms in patients • ECHO features include increased RV dimensions, RV regional wall motion
Interventional/surgical with severe mitral regurgitation abnormalities and dysfunction
• Mitral regurgitation (MR) • Heart transplantation or left ventricular assist devices may be required in • RV angiography has often been considered the gold standard for diagnosis,
• Radiofrequency catheter ablation for refractory AF patients • Systolic anterior motion (SAM) of the anterior mitral severe cases but cardiac MRI has better sensitivity and specificity
with HOCM valve leaflet • ECG-V1–3 abnormalities (typically T wave inversion), epsilon wave may be
• Implantable cardioverter defibrillator (ICD) implantation for • Asymmetric hypertrophy (ASH) present (described as terminal notch buried in the QRS complex)
primary prevention for those at risk of sudden death Restrictive cardiomyopathy
• Septal myectomy or alcohol septal ablation to reduce left Cardiac MRI Management
ventricular outflow gradient Definition
• Heart transplantation may be necessary in patients with • Can measure the severity and distribution of LVH, and • In ARVC, severe RV dysfunction is treated with standard heart failure
refractory heart failure provide information on systolic and diastolic ventricular Restrictive cardiomyopathy is a condition characterised by normal left medications
function ventricular cavity size and systolic function but with increased myocardial • Cardiac transplantation is considered if treatment is refractory
Genetic counselling stiffness. • Beta blockers are used in asymptomatic patients and an ICD is
Genetic testing recommended in high-risk patients
Careful pedigree analysis of family members can be useful in Causes
identifying those at risk of inheriting the disease; 1st-degree • Genetic mutations can be identified in approximately
relatives of patients with HOCM should be regularly screened 60% of patients. • Idiopathic (most common)
with ECG and echocardiography • Endomyocardial fibrosis (associated with Löffler syndrome)

18 / Chapter 1 Cardiology: Hypertrophic obstructive cardiomyopathy Mind Maps for Medicine \ 19

 • Stage 1 HTN Clinic BP • Hypertensive urgency A clinical Primary (essential) HTN Symptoms Signs of end-organ
≥140/90mmHg, or ambulatory situation in which BP is very high
damage:
BP monitoring (ABPM) daytime (e.g. ≥180/≥120mmHg) with no • No direct underlying cause (accounts for about 95% of • Usually asymptomatic
average/home BP monitoring signs or symptoms indicating • Signs of retinopathy:
cases) • Headaches
(HBPM) average ≥135/85mmHg acute organ damage grade 1 (silver or copper
• Linked to genetics, low birthweight, obesity, excessive • Visual disturbance
• Stage 2 HTN Clinic BP • Hypertensive emergency wiring), grade 2 (A-V
alcohol, excessive salt and metabolic syndrome • Dizziness
Hypertension (HTN) is persistently raised ≥160/100mmHg, or ABPM (or malignant HTN) Severe HTN nipping), grade 3 (flame
• Seizures
arterial blood pressure (BP). It is one of daytime average/HBPM average with potentially life-threatening Secondary HTN haemorrhages and cotton
the most common conditions and one BP ≥150/95mmHg symptoms and signs indicative of Signs wool spots), grade 4
of several risk factors for diseases such as • Severe 3 HTN Clinic systolic acute impairment of one or more This is secondary to a specific underlying cause: (papilloedema)
heart failure, myocardial infarction, stroke BP ≥180mmHg, or clinic diastolic organ systems • Vascular Coarctation of the aorta, renal artery stenosis • Signs of heart failure
• Renal Chronic pyelonephritis, diabetic nephropathy, Signs of underlying disease: • Signs of renal failure
and chronic kidney disease. BP ≥120mmHg
glomerulonephritis, polycystic kidney disease, renal cell • Risk factors e.g. obesity e.g. proteinuria
carcinoma • Signs of Cushing syndrome
Definition • Endocrine Primary hyperaldosteronism, • Radiofemoral delay or weak
femoral pulses (coarctation
Classification phaeochromocytoma, Cushing syndrome, Liddle
syndrome, congenital adrenal hyperplasia, acromegaly, of aorta)
thyroid disease • Renal bruits (renovascular disease)
• Drugs COCP, NSAIDs, corticosteroids • Palpable kidney
• Pregnancy Either as gestational HTN or pre-eclampsia
Clinical features
Aetiology

Hypertension

Investigations

Complications Management
• Coronary heart disease
• Stroke Confirming hypertension Looking for secondary causes
• Peripheral vascular disease
• Retinopathy To avoid ‘white coat’ hypertension, patients with stage 1 HTN should have For young patients (<40) with HTN or secondary
• Aortic aneurysm Long term Acute severe HTN one of the following to confirm hypertension: causes suspected:
• Heart failure • 24-h ambulatory BP At least two measurements per hour during the
• Thyroid function tests (exclude thyroid disease)
• Chronic kidney disease Lifestyle advice: • Severe HTN (e.g. systolic BP person’s usual waking hours (for example, between 08:00 and 22:00);
• 24-h urinary metanephrines (exclude phaeochromocytoma)
• Vascular dementia >200mmHg and/or diastolic the average value of at least 14 measurements should be used
• Salt restriction • Urinary free cortisol (exclude Cushing syndrome)
BP >120mmHg or signs of • Home readings For each BP recording, two consecutive
• Caffeine restriction • Renin/aldosterone level (exclude primary
target organ dysfunction e.g. measurements need to be taken, at least 1 min apart (with person
• Alcohol restriction hyperaldosteronism)
encephalopathy) is a medical seated); BP should be recorded twice daily, ideally in the morning and
• Smoking cessation • U&Es (↓K+ in primary hyperaldosteronism; impaired
emergency evening; BP should be recorded for at least 4 days (ideally 7 days); the
• Exercise creatinine/eGFR in renal disease)
• BP should be reduced gradually first day recording should be discarded and the average should be
• Lose weight • Plasma calcium (exclude hyperparathyroidism)
(over 24–48h) as rapid reduction taken of all the other values
• Eating healthy balanced diet • Renal ultrasound (exclude renal disease e.g. polycystic
in BP can result in stroke or MI kidneys)
• Medications of choice in Checking for end-organ damage
Pharmacological (see Notes) • MRI renal arteries (exclude renal artery stenosis)
this situation are IV sodium
• Examination of the fundi for the presence of hypertensive retinopathy
nitroprusside, IV labetolol
• Urine dipstick/microscopy to detect proteinuria and haematuria Assess cardiac risk profile
or GTN infusion
• Urine albumin: creatinine ratio (ACR) to detect microalbuminuria
• Continuous BP monitoring via an • Fasting blood glucose/HbA1c
• Serum U&Es to check for renal disease
arterial line and admission to high • Fasting lipid profile
• 12-lead ECG to look for left ventricular hypertrophy or signs of
dependency unit (HDU) or ITU
ischaemic heart disease
may be required
• ECHO to look for left ventricular hypertrophy/signs of heart failure

20 / Chapter 1 Cardiology: Hypertension Mind Maps for Medicine \ 21

 Hypertension notes
Pharmacological options

Diagnosis of ACE inhibitors • Include ramipril, lisinopril, perindopril

hypertension • Inhibit the conversion angiotensin I to angiotensin II and therefore the activation of the RAAS system
Clinic reading ≥ 140/90mmHg
• 1st-line treatment in younger patients (<55 years) and diabetic patients with hypertension
• Side effects include dry cough, angioedema and hyperkalaemia
• Renal function must be checked 2–3 weeks after starting due to the risk of worsening renal function in patients
Offer ABPM or HBPM with renovascular disease (acceptable changes are an increase in serum creatinine up to 30% from baseline
and an increase in K+ up to 5.5mmol/L)

<135/85 mmHg ≥135/85 mmHg ≥150/95 mmHg

Angiotensin receptor • Include losartan, candesartan, valsartan
blockers (ARBs) • Block effects of angiotensin II at the AT1 receptor and therefore the activation of the RAAS system
Stage 1 hypertension Stage 2 hypertension • Angiotensin II receptor blockers are generally used in situations where patients have not tolerated
Not hypertensive
an ACE inhibitor, usually due to the development of a cough
Monitor Treat if < 80 years AND any of the following: Treat all patients, • Side effects similar to ACE inhibitors (minus cough)
• target organ damage regardless of age • Like ACE inhibitors, renal function must be checked 2–3 weeks after starting due to the risk of worsening
• established cardiovascular disease renal function in patients with renovascular disease
• renal disease
• diabetes
• 10-year cardiovascular risk equivalent to Calcium channel blockers • Include amlodipine (most commonly used), nifedipine
10% or greater • Block voltage-gated Ca2+ channels relaxing vascular smooth muscle and the force of myocardial contraction
• 1st-line treatment in older patients (≥55 years)
• Common side effects include headache, flushing and ankle swelling

Thiazide diuretics • Include indapamide (thiazide of choice), chlorthalidone, bendroflumethiazide (no longer favoured by NICE)
Offer drug treatment for hypertension • Thiazides inhibit reabsorption of Na+ and Cl– from the distal convoluted tubules in the kidneys, by blocking
the NaCl symporter
• A step 3 treatment for patients who are already on ACE inhibitors/ARBs and Ca2+ channel blockers
• Common side effects include hyponatraemia, hypokalaemia and dehydration
Pharmacological
step-wise approach Spironolactone • An aldosterone antagonist, relatively weak diuretic with K+-sparing action
≥55y and no T2DM or
< 55 years Black African or African- • Used as step 4 treatment in patients with K+ <4.5mmol/L
• BP targets • Main side effects are hyperkalaemia and gynaecomastia
Reduce and maintain BP to the Caribbean ethnicity
following targets:
• Age <80y: Beta blockers • Include bisoprolol, carvedilol, atenolol, propranolol
• Clinic BP <140/90mmHg • Competitive antagonists that block the receptor sites for adrenaline and noradrenaline on adrenergic
Step 1 A C
• ABPM/HBPM beta receptors
<135/85mmHg • Used as step 4 treatment where patients are intolerant or contraindicated to using diuretics
• Age ≥80y:
• Clinic BP <150/90mmHg A + C or A + D C + A or C + D
• ABPM/HBPM Step 2 Alpha blockers • Include doxazosin (alpha-1 selective alpha blocker), methyldopa (alpha-2 adrenergic receptor agonist),
<145/85mmHg moxonidine (alpha-2/imidazoline receptor agonist)
• Postural hypertension: • Alpha-1 adrenergic receptor blockers inhibit the binding of noradrenaline to the alpha-1 receptors on vascular
• Base target on standing BP Step 3 A+C+D Key smooth muscle cells, resulting in vasodilatation, which decreases peripheral vascular resistance, leading to
• Frailty or multimorbidity: decreased BP
• Use clinical judgement A=A
 CE inhibitor or • Used as add-on therapy if above measures have failed to control BP or are not tolerated/contraindicated
Angiotensin 2
Step 4 receptor blocker
If K+ ≤ 4.5mmol/L add spironolactone Direct renin inhibitors • Include aliskiren
C = Calcium
If K+ > 4.5mmol/L, add an alpha- or beta-blocker channel blocker • Relatively newer drug which works by inhibiting renin and thus blocks the conversion of angiotensinogen
to angiotensin I
If BP not controlled on 4 drugs then seek specialist review D = t hiazide-like • Can be used alone or in combination with other antihypertensives
Diuretic • Initial trials suggest aliskiren reduces BP to a similar extent as ACE inhibitors or ARBs
• Main side effect is diarrhoea

22 / Chapter 1 Cardiology: Hypertension notes Mind Maps for Medicine \ 23

 • IE typically develops on the valvular • Staphylococcus aureus (most common Symptoms
surfaces of the heart that have cause overall)
sustained endothelial damage • Streptococci: most commonly viridans • Fever
secondary to turbulent blood flow streptococci • Rigors
• A mass of fibrin, platelets and infectious • Coagulase-negative staphylococci • Night sweats
organisms form vegetations along the (account for 30% of cases of IE in • Malaise
edges of the valve (see Fig. 1) prosthetic valves) • Weight loss
Infective endocarditis (IE) is an • The valves most commonly affected • Pseudomonas aeruginosa • Loss of appetite
infection of the endocardium of by IE are (in decreasing order of • HACEK organisms: Haemophilus • Fatigue Fig. 2 Splinter haemorrhage
the heart usually involving the frequency): 1 mitral valve, 2 aortic valve, spp., Aggregatibacter spp., • Flu-like illness
heart valves. It may occur as a 3 combined mitral and aortic valve, Cardiobacterium spp., Eikenella • Joint pain
fulminating or acute infection but 4 tricuspid valve (more common in IV Fig. 1 Acute infective endocarditis causing corrodens and Kingella spp.
more commonly runs an insidious drug users), pulmonary valve (rare) vegetations along the mitral valve • Enterococci Signs
course which is known as subacute • Fungi
endocarditis. • Fever
Pathophysiology •
•
Heart murmurs
Petechiae
Causative organisms • Splinter haemorrhages
Definition (see Fig. 2) Fig. 3 Clubbing
• Osler nodes
• Janeway lesions
• Clubbing (see Fig. 3)
• Arthritis
• Splenomegaly
Clinical features • Meningism
• Roth spots (see Fig. 4)
Fig. 4 Roth spot

Infective endocarditis

Diagnostic criteria Risk factors

Duke’s criteria of IE (must meet 2 major criteria, or 1 major and 3 minor • Valvular heart disease with stenosis
criteria, or 5 minor criteria): or regurgitation
• Valve replacement
Major criteria • Structural congenital heart disease
Complications Management Investigations • Previous IE
• Positive blood culture: • Hypertrophic cardiomyopathy
• Typical micro-organism for IE from 2 separate blood cultures or • Recreational drug abuse
• MI, pericarditis, cardiac • Antibiotics Give IV for first 2 weeks • Bloods FBC (↓Hb, ↑WCC), CRP/ESR (↑), U&Es • Persistently positive blood cultures • Invasive vascular procedures
arrhythmias and orally for further 2–4 weeks; (possible AKI), serum immunoglobulins (↑), • Evidence of endocardial involvement:
• Heart valve insufficiency empirical antibiotic therapy should complement levels (↓) • Oscillating intracardiac mass on valve/supporting structures or
• Congestive cardiac failure be given while awaiting blood • Blood cultures 3 sets taken at different sites and • Abscess or
• Sinus of Valsalva aneurysm cultures, usually a combination of different times • New partial dehiscence of prosthetic valve or new valvular regurgitation
• Aortic root or myocardial abscess IV benzylpenicillin and gentamicin • Urinalysis Microscopic haematuria
• Arterial emboli, infarctions, (local hospital protocol should be • ECG 10% of patients will develop conduction defects Minor criteria
mycotic aneurysms followed and advice sought from a • CXR Possible heart failure or evidence of septic emboli
• Arthritis, myositis microbiologist); subsequent treatment in right-sided endocarditis • Predisposing heart condition/IV drug use
• Glomerulonephritis, acute kidney depends on cultures and sensitivity • ECHO Transthoracic ECHO should be performed within • Fever (>38°C)
injury • Surgery Valve repair or valve 24h; identifies vegetations and underlying valvular • Vascular phenomenon Major arterial emboli, septic pulmonary infarcts,
• Stroke replacement abnormalities mycotic aneurysm, intracranial haemorrhage, conjunctival haemorrhage,
• Mesenteric or splenic abscess or • Serology Useful if unusual organisms are suspected Janeway lesions
infarction e.g. Coxiella spp., Bartonella spp. or Legionella spp. • Immunological phenomenon Glomerulonephritis, Osler nodes,
• MRI, nuclear imaging and multi-slice CT coronary Roth spots, rheumatoid factor
angiography are other imaging modalities which • Positive blood cultures Not meeting major criteria
may be used • ECHO Consistent with IE but not meeting major criterion

24 / Chapter 1 Cardiology: Infective endocarditis Mind Maps for Medicine \ 25

 Chapter 2
02
Respiratory

Acute respiratory distress syndrome................................................................................................................28

Asthma................................................................................................................................................................................30
Bronchiectasis.................................................................................................................................................................34
Chronic obstructive pulmonary disease........................................................................................................36
Cystic fibrosis...................................................................................................................................................................40
Interstitial lung disease.............................................................................................................................................42
Lung cancer.....................................................................................................................................................................46
Obstructive sleep apnoea.......................................................................................................................................50
Pleural effusion..............................................................................................................................................................52
Pneumonia.......................................................................................................................................................................54
Pneumothorax...............................................................................................................................................................56
Pulmonary embolism................................................................................................................................................60
Respiratory failure........................................................................................................................................................62
Sarcoidosis........................................................................................................................................................................64

Mind Maps for Medicine \ 27

 • Sepsis (most common)
• Trauma
• Hypovolaemic shock
• Acute inflammation or injury at the • This loss also impairs compensatory • Pneumonia
Acute respiratory distress syndrome lung’s alveolar-capillary membrane pulmonary vasoconstriction in • Gastric aspiration
(ARDS) is an acute life-threatening, increases the permeability of the response to hypoxia • Fat or amniotic fluid embolism • Bloods FBC, U&Es, LFTs, amylase,
diffuse, inflammatory form of lung pulmonary microvasculature causing • The combination of these two • Acute pancreatitis clotting, CRP, blood cultures
injury that is associated with a leakage of fluid across the membrane processes causes profound • Burns • Cyanosis • ABG Type 1 respiratory failure
variety of causes (but often caused • The resulting acute inflammatory hypoxaemia in association with high • Smoke inhalation • Tachypnoea • CXR Bilateral alveolar shadowing,
by infection). It is characterised exudate inactivates surfactant leading permeability pulmonary oedema • Cardiopulmonary bypass • Tachycardia often with air bronchograms
by increased lung microvascular to collapse and consolidation of distal • The predominant cause of death in • Diabetic ketoacidosis • Bilateral fine inspiratory crackles (see Fig. 1)
permeability, resulting in lung parenchyma with progressive ARDS is multiple organ failure
hypoxaemic respiratory failure. loss of lung gas exchange surface area
Risk factors Clinical features Investigations
Definition Pathophysiology

Acute respiratory distress syndrome

Notes
Acute respiratory distress syndrome

Diagnostic criteria

Requires all 3 to exist:

• Acute onset: 20–50% of acute lung injury patients will develop ARDS
Management within 7 days
• CXR shows bilateral infiltrates (Fig. 1)
• Refractory hypoxaemia: PaO2:FiO2 <200

Admit to ITU, give supportive therapy (see below) and treat

the underlying cause

Respiratory support

• Prone positioning can improve oxygenation

• CPAP Can be used to treat early ARDS
• Ventilation Indications for ventilation include PaO2
<8.3 despite 60% FiO2 or a PaCO2 >6kPa

Circulatory support

• Invasive haemodynamic monitoring with an arterial

line and Swan–Ganz catheter to monitor pulmonary
capillary wedge pressure and cardiac output
• IV fluids
• Inotropes e.g. dobutamine
• Vasodilators e.g. low-dose nitric oxide

Fig. 1 ARDS on CXR

28 / Chapter 2 Respiratory: Acute respiratory distress syndrome Mind Maps for Medicine \ 29
 • Atopic asthma is the result of airway inflammation caused by exposure to an • Personal history of atopy Triggers Symptoms
environmental allergen e.g. eczema and hay fever
• Patients with asthma have an exuberant Th2-mediated Ig immune response • Family history of asthma or atopy • Respiratory infections • Intermittent SOB
Asthma is a chronic inflammatory • IgE binds to bronchial mast cells resulting in degranulation and the release • Inner city environment, socio- • Cold air • Wheeze: polyphonic
condition of the airways characterised of pro-inflammatory mediators economic deprivation • Exercise • Cough: often nocturnal ± sputum
by hyper-responsiveness and • There are 2 phases of inflammation: • Obesity • Pollution e.g. cigarette smoke,
constriction in response to a • Acute phase Characterised by bronchoconstriction and airway oedema; • Prematurity and low birth weight fumes Signs
variety of stimuli. Narrowing of the this process begins within minutes of allergen exposure and resolves • Viral infections in early childhood • Allergens e.g. pollen, dust mite,
airways is usually reversible (either within hours animals • ↓Chest expansion
• Smoking
spontaneously or with medication) • Delayed phase Pro-inflammatory mediators, such as IL-5, released by • Time of day • Bilateral polyphonic wheeze
• Maternal smoking
leading to intermittent symptoms, mast cells recruit eosinophils, basophils and Th2 lymphocytes, resulting in • Work-related (occupational) • Tachypnoea
• Early exposure to broad-
but in some people with chronic ongoing inflammation, sensitisation of sensory nerve endings, resulting • Drugs e.g. beta blockers and • Tachycardia
spectrum antibiotics
asthma, the inflammation may lead in bronchial hyper-responsiveness NSAIDs • Reduced air entry
to irreversible airflow obstruction. • Emotional factors e.g. stress, • Hyperinflated chest
laughter
• Gastro-oesophageal reflux
Definition Pathophysiology disease Clinical features
Risk factors

• Pneumonia
• Pneumothorax
Complications • Pneumomediastinum
• Respiratory failure and arrest
• Pulmonary collapse

Asthma
Notes
Asthma

Investigations

Acute asthma Chronic asthma

• Bloods FBC: raised WCC and typically Spirometry: FeNO:

eosinophilia; U&Es: salbutamol can The bronchodilator reversibility (BDR) test The FeNO (fractional exhaled nitric oxide) test is a
cause hypokalaemia; CRP: to rule out is preferred over peak flow measurement relatively new test which measures the level of NO in the
infection for initial confirmation of obstruction exhaled breath and provides an indication of eosinophilic
• ABG Patients with SpO2 <92% or other of airways in the diagnosis of asthma. inflammation in the lungs; in adults a level of ≥40 parts
features of life-threatening asthma An FEV1/FVC ratio <70% is considered per billion (ppb) is considered positive.
require ABG measurement obstructive. A positive bronchodilator
• CXR To rule out pneumonia/ reversibility test is indicated by an
pneumothorax; typically normal or improvement in FEV1 of 12% or more and
shows hyperinflation and flattened increase in volume of 200ml or more.
diaphragm (see Fig. 2)
• Peak flow Peak expiratory flow (PEF)
expressed as a % of the patient’s
Peak flow:
previous best value is most useful Measurement of peak expiratory flow
clinically; in the absence of this, rate (PEFR) via a peak flow meter (see
PEF as a % of predicted is also useful Fig. 1) is the simplest and most basic
• ECG Often sinus tachycardia test for diagnosing asthma; diurnal Fig. 2 CXR showing hyperinflation
Fig. 1 Peak flow meter
variation typically in the early morning is
suggestive of asthma. However, PEFR is
more useful in the monitoring of patients
with established asthma.

30 / Chapter 2 Respiratory: Asthma Mind Maps for Medicine \ 31

 Asthma notes Notes
Asthma

Grading severity of asthma attack Management of chronic asthma: stepwise approach

Moderate Severe Life-threatening Near fatal

Step Details
• PEFR 50–75% best or predicted • PEFR 33–50% best or predicted • PEF <33% best or predicted • Raised PaCO₂ and/or requiring
1. Newly diagnosed asthma Short-acting beta agonist (SABA)
• Speech normal • Can’t complete sentences • SpO₂ <92% mechanical ventilation with
i.e. salbutamol PRN
• RR <25/min • RR >25/min • PaO₂ <8kPa raised inflation pressures
• Pulse <110bpm • Pulse >110bpm • ‘Normal’ PaCO₂ (4.6–6.0kPa)
2. Not controlled on step 1 SABA + low-dose* inhaled
• Altered conscious level
OR corticosteroid (ICS) e.g.
• Exhaustion
newly diagnosed asthma with beclomethasone or budesonide
• Arrhythmia
symptoms ≥3/week or night-
• Hypotension
time waking
• Cyanosis
• Silent chest
3 SABA + low-dose ICS + leukotriene
• Poor respiratory effort
receptor antagonist (LTRA) e.g.
montelukast 10mg OD

4 • SABA + low-dose ICS + long-

acting beta agonist (LABA)
e.g. formoterol
Management of acute asthma • Continue LTRA depending on
patient’s response to LTRA

5 • SABA ± LTRA
Sit patient up and high-flow oxygen
• Switch ICS/LABA for a
maintenance and reliever
therapy (MART) that includes
a low-dose ICS e.g. symbicort
(budesonide with formoterol)
Nebulised beta-2 agonist (salbutamol 5mg) and
nebulised antimuscarinic (ipratropium bromide 0.5mg) 6 • SABA ± LTRA + moderate-
dose** ICS MART or
• Consider changing back to a
fixed dose of a moderate-dose
ICS and a separate LABA
Corticosteroid: prednisolone (40–50mg) or IV hydrocortisone (100mg)
7 SABA ± LTRA + one of the following
options:
• Increase ICS to high-dose***
(only as a fixed-dose regime,
Consider IV magnesium sulphate (1.2–2.0g IV over 20min) not as part of MART)
if little response to above IV aminophylline can also be considered • Trial an additional drug such as a
long-acting muscarinic receptor
antagonist e.g. tiotropium or
theophylline
• Seek advice from a healthcare
ITU referral for consideration of ventilation support for those who are failing to respond professional with expertise in
to therapy such as deteriorating PEF, persisting or worsening hypoxia, hypercapnia, asthma
respiratory acidosis, exhaustion, feeble respiration, drowsiness, confusion,
altered conscious state, respiratory arrest *Low dose ≤400µg budesonide or equivalent
**Moderate dose 400–800µg budesonide or equivalent
***High dose >800µg budesonide or equivalent

32 / Chapter 2 Respiratory: Asthma notes Mind Maps for Medicine \ 33

 • This is dependent on the cause; the most common cause is infection Congenital Symptoms
Bronchiectasis is a permanent • Infection of the small distal airways results in inflammation and release of
dilatation and thickening of the inflammatory mediators • Kartagener’s syndrome (primary ciliary dyskinesia) Impaired • Persistent cough
airways characterised by chronic • This impairs ciliary action resulting in bacterial proliferation and tissue ciliary mobility resulting in ↑susceptibility to infection; results in • Copious purulent sputum
cough, excessive sputum production, damage which cause bronchial dilatation situs inversus • Intermittent haemoptysis
bacterial colonisation and recurrent • The most common organisms isolated from patients with bronchiectasis • Alpha-1 antitrypsin (A1AT) deficiency • Fever
acute infections. It is caused by include: Haemophilus influenzae (most common), Pseudomonas aeruginosa, • Yellow nail syndrome Associated with azoospermia • Malaise
chronic inflammation of the airways Klebsiella pneumoniae and Streptococcus pneumoniae • Cystic fibrosis (see Ch2: Cystic fibrosis)
and is associated with, or caused by, Signs
a large number of diseases. It may Acquired
be widespread throughout the lungs Pathophysiology • Finger clubbing (see Fig. 1)
• Post infection Childhood respiratory infections e.g. measles, • Coarse inspiratory crackles Fig. 1 Finger clubbing
(diffuse) or more localised (focal).
pertussis, TB, bacterial pneumonia • Wheeze
• Immunodeficiency Including HIV
Definition • Connective tissue disorders e.g. RA, SLE, Sjögren syndrome
• Asthma
• Allergic bronchopulmonary aspergillosis Clinical features
• Gastric aspiration
• Bronchial obstruction Tumour, lymphadenopathy, foreign body
• Inflammatory bowel disease

Causes

Bronchiectasis
Investigations

• Imaging:
• CXR May be normal or show ring or tubular opacities, tramlines
and fluid levels
• High-resolution CT The diagnostic gold standard (see Fig. 2)
• Sputum microbiology To identify causative organisms
• Routine bloods FBC (↑WCC in presence of infection), polycythaemia
(in advanced cases), CRP (↑ in presence of infection)
Management • Immunological tests Serum immunoglobulins (IgG, IgA, IgM) and serum
Complications electrophoresis; serum IgE, skin prick testing/serum IgE testing to Aspergillus
fumigatus and aspergillosis precipitins
• Lung function tests Often show an obstructive pattern; reversibility
Conservative Medical Surgical • Repeated infections should be assessed
• Empyema • Bronchoscopy To locate the site of haemoptysis or exclude obstruction
• Physical training (e.g. inspiratory • Antibiotics For exacerbations • Lung resection surgery • Testing for cystic fibrosis (see Ch2: Cystic fibrosis)
• Lung abscess
muscle training) and chest (empirical while awaiting Considered for localised disease
• Pneumothorax
physiotherapy sensitivities) + long-term rotating when symptoms are not
• Life-threatening haemoptysis
• Postural drainage should be antibiotics in severe cases controlled by medical treatment
• Respiratory failure
performed regularly • Bronchodilators For patients • Bronchial artery embolisation/
• Right heart failure
who have a degree of reversibility surgery 1st line for management
• Cerebral abscess
• Immunisation Against influenza of massive haemoptysis
• Amyloidosis (rare)
and pneumococcus • Lung transplantation
• Reduced quality of life
Considered for end-stage disease
if pulmonary function is very poor
(FEV1 <30% of predicted)

Fig. 2 Distortion of lung parenchyma caused by cystic

bronchiectasis with predominant involvement of the lower lobes

34 / Chapter 2 Respiratory: Bronchiectasis Mind Maps for Medicine \ 35

Chronic obstructive pulmonary disease (COPD) is Chronic bronchitis Symptoms
characterised by airflow obstruction that is not fully
reversible. The airflow obstruction does not change • Airflow limitations are seen mainly in the small airways • Breathlessness
markedly over several months and is usually progressive • Tobacco smoking About 90% of cases are caused by caused by inflammation, narrowing and inflammatory • Cough: usually worse in the mornings and productive of small amount
in the long term. COPD is predominantly caused by cigarette smoking exudates of colourless sputum
smoking. COPD consists of two subtypes which may • Occupational exposure Dust, chemicals, noxious gases • There is an increased number of goblet cells and size • Wheeze: may occur typically on exertion or in exacerbations
occur independently or together: and particles (such as coal, grains, silica, welding fume, of bronchial submucosal glands resulting in mucous
• Chronic bronchitis Defined clinically as cough isocyanates and polycyclic aromatic hydrocarbons) have hypersecretion; this causes bronchial wall narrowing Signs
productive of sputum for at least 3 months in each year been associated with the development of COPD • This is compounded by ciliary dysfunction caused by
for 2 consecutive years • Air pollution Indoor and outdoor air pollution may squamous metaplasia of the epithelium; this overall • Tachypnoea
• Emphysema Enlargement of the air spaces distal contribute to the development of COPD causes bronchial wall narrowing • Breathlessness on exertion
to the terminal bronchioles in the lungs, either from • Genetics Homozygous alpha-1antitrypsin (A1AT) • Increased use of accessory muscles of respiration
dilatation, destruction or distension of their walls deficiency accounts for less than 1% of COPD cases Emphysema • Pursed lip breathing
• Cyanosis
• Alveolar walls are destroyed resulting in bullae • Wheeze
Definition Causes formation and fusion of adjacent alveoli
• This results in reduced exchange, decreased elastic
•
•
Hyperinflation (barrel chest)
Abnormal posture: patients may lean forward and rest their arms
recoil, progressive air trapping and hyperinflation on the table to ease breathing
• May be caused by A1AT deficiency • Drowsiness, flapping tremor and mental confusion (↑CO2)
• Signs of cor pulmonale: peripheral oedema, raised JVP

Pathophysiology
Clinical features
Chronic obstructive pulmonary disease

• Acute exacerbations ± infections

Complications • Polycythaemia
• Respiratory failure (type 2)
Investigations • Cor pulmonale: right-sided heart
failure 2° to chronic pulmonary
hypertension
Management • CXR To explore other diagnoses – may be normal • Pneumothorax
or show hyperinflation, flattened diaphragms, bullae • Lung cancer
(see Fig. 1)
• Bloods FBC (in the chronic setting) to exclude
anaemia and polycythaemia; eosinophils may be
Acute Chronic
raised if asthmatic component present; in the acute
• Controlled O2 therapy Start at 24–28% via venturi • General management Smoking cessation advice; annual influenza setting, WCC may be raised if underlying infection,
mask and increase accordingly (aim for O2 saturations vaccination + one-off pneumococcal vaccination CRP is useful acutely to identify infection; U&Es, A1AT
of 88–92%) • Inhaler therapy Short-acting beta-2 agonist (SABA), short-acting in younger patients, or in those who are not exposed
• Nebulised bronchodilators Salbutamol 5mg and muscarinic antagonist (SAMA), long-acting beta-2 agonist (LABA) and to cigarette smoke
ipratropium bromide 500µg; repeat as required inhaled corticosteroids (see Notes) • Sputum Microscopy, culture and sensitivity if
• Steroids Oral prednisolone 40mg, or 200mg IV • Oral theophylline NICE recommends theophylline only after trials of short- suspected infection
hydrocortisone and long-acting bronchodilators or those who cannot use inhaled therapy • ABG Typically shows type 2 respiratory failure; very
• Antibiotics If evidence of infection • Mucolytics Should be ‘considered’ in patients with a chronic productive important to guide O2 therapy and need for non-
• Consider IV aminophylline or salbutamol if there is cough invasive ventilation
inadequate response to nebulisers • Long-term O2 therapy (LTOT) Offer to patients with a PO2 of <7.3kPa • ECG May show signs of cor pulmonale (P pulmonale,
• Non-invasive positive pressure ventilation (NIPPV) or to those with a PO2 of 7.3–8kPa and one of the following: secondary right ventricular hypertrophy, right bundle branch
If no response to above and if respiratory rate >30 or polycythaemia, nocturnal hypoxaemia, peripheral oedema, pulmonary block)
pH <7.35; settings are guided by arterial blood gases hypertension • Spirometry Aids diagnosis to demonstrate airflow
• Consider ITU, intubation and ventilation if inadequate • Prophylactic antibiotic e.g. Azithromycin prophylaxis recommended obstruction: FEV1/FVC ratio less than 70%; also used
response to above in select patients to determine severity (see Notes)
• Pulmonary rehabilitation • CT To investigate abnormalities seen on CXR,
• Surgery Resection of large bullae or lung volume reduction surgery may confirm emphysematous bullae
• ECHO To assess cardiac status if features of cor
pulmonale Fig. 1 COPD with marked hyperinflation
and flattened diaphragms

36 / Chapter 2 Respiratory: Chronic obstructive pulmonary disease Mind Maps for Medicine \ 37
 Chronic obstructive pulmonary disease notes

Severity of COPD by spirometry Stepwise inhaler therapy

The severity of COPD is

categorised using FEV1: Post-bronchodilator FEV1/FVC FEV1 (of predicted) Severity
A short-acting beta-2 agonist (SABA) or short-acting muscarinic
<0.7 ≥80% Stage 1: Mild* antagonist (SAMA)

<0.7 50–79% Stage 2: Moderate

<0.7 30–49% Stage 3: Severe

<0.7 <30% Stage 4: Very severe Remain breathless or have exacerbations

*Symptoms should be present to diagnose COPD in these patients

Severity of COPD by clinical features

*Asthma features/steroid responsiveness features?
For this the Medical Research
Council (MRC) dyspnoea scale MRC dyspnoea scale
is used:
Grade 1 Not troubled by breathlessness except on strenuous exertion

Grade 2 Short of breath when hurrying on level ground or walking up a slight incline Asthma features/steroid responsiveness features No asthma features/steroid-responsive features

Grade 3 Walks slower than contemporaries because of breathlessness, or has to stop for breath when
walking at own pace

Grade 4 Stops for breath after walking about 100m or stops after a few minutes of walking on level ground Add a LABA + long-acting muscarinic
Consider a long-acting beta-2 agonist (LABA)
antagonist (LAMA) (if already taking a SAMA,
Grade 5 Too breathless to leave the house or breathless on dressing or undressing + inhaled corticosteroid (ICS)
discontinue and switch to a SABA)

Remain breathless or have exacerbations Remain breathless or have exacerbations

Offer triple therapy (i.e. LAMA + LABA + ICS);

if already taking a SAMA, discontinue Consider triple therapy (i.e. LAMA + LABA + ICS)
and switch to a SABA

*Determining whether a patient has asthmatic/steroid-responsive features:

• Any previous secure diagnosis of asthma or atopy
• A higher blood eosinophil count
• Substantial variation in FEV1 over time (at least 400ml)
• Substantial diurnal variation in peak expiratory flow (at least 20%)

38 / Chapter 2 Respiratory: Chronic obstructive pulmonary disease notes Mind Maps for Medicine \ 39
 • The defective protein is a cAMP-regulated Cl– channel, Respiratory/ENT
Cystic fibrosis (CF) is an autosomal CFTR, whose gene is on the long arm (q) of chromosome 7 Notes
recessive disorder and the most Cystic fibrosis
• There are many types of defect in the CFTR gene, of which • Cough with purulent sputum
common inherited condition in the most common, a deletion of phenylalanine at position • Wheeze
Caucasian individuals. It is due 508, accounts for about 70% of the total • Recurrent chest infections (organisms which
to a defect in cystic fibrosis • The abnormal CFTR channel in the cell membrane leads to may colonise CF patients: Staphylococcus aureus,
transmembrane conductance the production of excessively viscid secretions in the body Pseudomonas aeruginosa, Burkholderia cepacia,
regulator (CFTR), a chloride with a high concentration of Na+ and a low concentration • CF is the most common inherited Aspergillus spp.)
channel found in cells lining the of Cl– in exocrine secretions disease in Caucasians • Reduced exercise tolerance
lungs, intestines, pancreatic ducts, • As the Cl– channel is found in cells lining the lungs, • Prevalence is 1 in 2500 newborn • Nasal polyps
sweat glands and reproductive intestines, pancreatic ducts, sweat glands and reproductive infants • Sinusitis
organs. organs, it has various complications affecting these organs • Carrier frequency is 1 in 25 • Bronchiectasis
• Pneumothorax
• Respiratory failure
Definition Pathophysiology Epidemiology • Cor pulmonale

Gastrointestinal

• Pancreatic insufficiency (diabetes mellitus,

steatorrhoea)
• Meconium ileus (in neonates)
• Rectal prolapse (infants)
• Distal intestinal obstruction syndrome
• Gallstones
• Cirrhosis
Cystic fibrosis Miscellaneous

• Male infertility (congenital absence of vas deferens)

• Osteoporosis
• Arthritis
• Vasculitis
• Failure to thrive (neonates/children)
• Hypertrophic pulmonary osteoarthropathy (HPOA)
• Finger clubbing

Prognosis Management
Clinical features
• There is no current cure for CF
• Estimated survival for a child born now Conservative
is 40–50 years; most deaths are due
to respiratory failure • Multidisciplinary approach
• Females and those from lower • Regular (at least twice daily) chest physiotherapy and postural drainage
socioeconomic classes have a poorer • Deep-breathing exercises Investigations
prognosis • High-calorie diet, including high-fat intake
• The use of recombinant human DNase • Routine vaccinations and annual flu jab • Antenatal screening or newborn screening test
has shown a marked improvement May pick up CF
Medical
in survival of patients with a low FEV1 • Sweat testing Confirms the diagnosis and is 98%
• Respiratory: inhaled or nebulised bronchodilators, prophylactic sensitive; Cl– >60mmol/L with Na+ < Cl– on two
antibiotics, recombinant DNase, nasal steroids for polyps, mucolytic separate occasions
agents; high-dose ibuprofen may slow progression of lung disease, • Molecular genetic testing CFTR gene
especially in children • Sinus X-ray or CT Opacification of the sinuses
• Liver disease: ursodeoxycholic acid is present in almost all patients with CF
• Pancreas: pancreatic enzyme supplements taken with meals, • CXR Hyperinflation and bronchiectasis changes
multivitamins, insulin • CT thorax To help diagnose bronchiectasis
• Bisphosphonates • Bloods FBC, U&Es, fasting glucose, LFTs and
vitamins A, D and E
Surgical • Lung function testing Spirometry is unreliable
in children <6 years
• Heart/lung transplant • Sputum microbiology To isolate common pathogens
• Liver transplant that cause chest infections
• Semen analysis (if appropriate)
40 / Chapter 2 Respiratory: Cystic fibrosis Mind Maps for Medicine \ 41
 Known causes

• Industrial dust diseases Including coal worker’s pneumoconiosis, silicosis, asbestosis and berylliosis
• Drugs Including nitrofurantoin, bleomycin, amiodarone and sulfasalazine
•
•
Hypersensitivity pneumonitis
Infections Fungal e.g. histoplasmosis; bacterial e.g. TB; or viral e.g. COVID-19
Hypersensitivity pneumonitis
Interstitial lung disease refers to a • Radiation Definition
group of chronic conditions which • Malignancy e.g. Metastases or lymphangitis carcinomatosis
produce interstitial lung damage • Paraquat poisoning Hypersensitivity pneumonitis, also known as extrinsic allergic alveolitis, is a
and fibrosis resulting in loss of condition caused by hypersensitivity-induced lung damage due to a variety
Associated with systemic inflammatory disorders of inhaled organic particles.
the elasticity of the lungs. It may
• Dyspnoea on exertion
be secondary to a wide range • Sarcoidosis
of diseases or may be idiopathic
• Cough (non-productive) Pathophysiology
• RA • Crepitations on auscultation
with no known underlying cause. • SLE (specific sounds depend on It is thought to be largely caused by immune-complex-mediated tissue
Pulmonary fibrosis can be localised, • Systemic sclerosis and mixed connective tissue disease underlying cause) damage (type III hypersensitivity) though a delayed hypersensitivity (type IV)
segmental, lobar, or affect the • Ankylosing spondylitis is also thought to play a role, particularly in the chronic phase.
entirety of the lung(s).
Idiopathic Clinical features Types

• Idiopathic pulmonary fibrosis (IPF) • Farmer’s lung One of the most common forms; due to exposure to mouldy
Definition • Cryptogenic organising pneumonia hay; the major antigen is Saccharopolyspora rectivirgula
• Acute interstitial pneumonia, desquamative interstitial pneumonia and respiratory bronchiolitis • Bird-fancier’s lung Caused by exposure to avian proteins e.g. pigeons
and parakeets
• Cheese-worker’s lung Caused by exposure to cheese mould,
Classification by cause Penicillium casei
• Malt worker’s lung Caused by exposure to Aspergillus clavatus in
mouldy malt
• Hot tub lung Caused by exposure to Mycobacterium avium in poorly
maintained hot tubs
• Chemical worker’s lung Due to exposure to trimellitic anhydride,
diisocyanate and methylene diisocyanate during the manufacture
Interstitial lung disease of plastics, polyurethane foam and rubber
• Mushroom worker’s lung Due to exposure to thermophilic
Investigations actinomycetes in mushroom compost

Clinical features/diagnosis

• Acute presentation (several hours after exposure to antigen) Fever,

malaise, cough, shortness of breath and coarse end-inspiratory crackles
Management • Chronic presentation Progressive exertional dyspnoea, weight loss
• CXR and high-resolution CT (see Fig. 1, Table 1) Features include
and cough
honeycombing, traction bronchiectasis, lung architectural distortion,
• Bloods may show raised WCC in acute phase and CXR typically shows
• Treat underlying cause if reticulation and interlobular septal thickening
upper/middle zone fibrosis
possible e.g. antibiotics for • Lung function tests Spirometry shows a restrictive pattern with
• Bronchoalveolar lavage may show lymphocytosis
infection, treat malignancy, reduced transfer factor for carbon monoxide
stop offending drugs, steroids • ABG May show type 1 respiratory failure Management
or immunosuppressant for • Lung biopsy May confirm diagnosis
autoimmune disorders • Allergy avoidance and may require change of job
• Avoid exposure to irritants e.g. Table 1 Causes of fibrotic shadowing on CXR depending on predominant location • Prednisolone may be used for treatment in severe cases
asbestosis, coal etc.
• Supportive treatment, Upper-zone Mid-zone Lower-zone
including oxygen therapy and predominant predominant predominant
pulmonary rehabilitation
• Claim compensation for • TB • Progressive • IPF
occupational-related illness • Hypersensitivity massive • Asbestosis
• Lung transplantation pneumonitis pulmonary fibrosis • Scleroderma
• Ankylosing • RA-associated
spondylitis interstitial disease Fig. 1 Pulmonary fibrosis on CXR, with CT inset
• Sarcoidosis
• Histoplasmosis
• Silicosis

42 / Chapter 2 Respiratory: Interstitial lung disease Mind Maps for Medicine \ 43

 Industrial dust diseases

Idiopathic pulmonary fibrosis (IPF) Coal worker’s pneumoconiosis (CWP) Silicosis Asbestosis
Definition Definition Definition Definition

IPF (previously termed cryptogenic fibrosing alveolitis) is a chronic lung A common dust disease in countries that have or have had underground coal Silicosis is a fibrotic lung disease caused by the inhalation of fine particles Asbestosis is a typical pneumoconiosis caused by inhalation of asbestos fibres.
condition characterised by progressive fibrosis of the interstitium of the lungs. mines. It results from inhalation of coal dust over years (approx. 15–20 years). of crystalline silicon dioxide (silica). Asbestos can cause a variety of other lung diseases including benign pleural
The term IPF is reserved for when no underlying cause exists. plaques, pleural thickening, asbestosis, mesothelioma and bronchial
Pathophysiology Pathophysiology adenocarcinoma.
Clinical features/diagnosis
• Tiny particles of coal dust (2–5µm diam.) are retained in the alveoli • Dust containing crystalline silica is highly fibrogenic Pathophysiology
• Highest incidence in late-middle age • They are engulfed by macrophages but, eventually, the system • When silica dust is inhaled, the particles deposit within the distal airways
• Progressive exertional dyspnoea is overwhelmed and an immune response follows; this produces • Macrophages ingest these particles and initiate an inflammatory response • The result of exposure to asbestos with blue asbestos (crocidolite) being
• Dry cough pulmonary fibrosis by releasing pro-inflammatory molecules leading to the formation of the most fibrogenic, white (chrystotile) being least and brown (amosite)
• Bibasal fine end-inspiratory crepitations on auscultation • Caplan syndrome When CWP is associated with RA, and pulmonary nodular lesions and tissue fibrosis being intermediate
• Clubbing rheumatoid nodules • Occupations at risk of silicosis include those who worked in mining, slate • The development of pulmonary fibrosis appears to be related to the severity
• CXR and CT Shows bilateral interstitial shadowing – typically small, irregular, works, foundries and potteries and duration of exposure; the latent period is typically 15–30 years
peripheral opacities (‘ground-glass’) later progressing to ‘honeycombing’ Clinical features/diagnosis
• ANA positive in approx. 30% and RF positive in approx. 10% Clinical features/diagnosis Clinical features/diagnosis
• Patients with simple CWP are often asymptomatic and incidentally found
Management on CXR although co-existing chronic bronchitis is common • Simple nodular silicosis Usually asymptomatic and may be incidentally • Most people have occupational exposure such as fitting or working with
• CXR in CWP shows many small round opacities (1–10mm) in the upper zones found on CXR asbestos insulation e.g. builders, plumbers and electricians
• Pulmonary rehabilitation • CWP may progress to progressive massive pulmonary fibrosis caused by • Advanced nodular silicosis Cough and exertional dyspnoea are common • It usually presents with progressive dyspnoea, dry cough, repetitive
• Treatment is limited: pirfenidone (an antifibrotic agent) may be useful continuous exposure; it is characterised by large fibrotic masses (1–10cm) • There is increased risk of TB and COPD inspiratory basal crackles and clubbing of the fingers (advanced feature)
in selected patients predominantly in the upper lobes • CXR Miliary or nodular pattern in upper and mid zones and thin streaks of • CXR shows diffuse bilateral shadowing and honeycomb lung; pleural
• Many patients will require oxygen therapy and eventually lung transplant • Symptoms of progressive massive pulmonary fibrosis include breathlessness, calcification are seen around the hilar lymph nodes (‘eggshell’ calcification) plaques may be present which are an indicator of previous exposure to
if suitable cough productive of black sputum and cor pulmonale asbestos
Management
Management Management
• There is no specific cure
• There is no specific treatment; patients should avoid exposure to coal dust • Management involves avoiding further exposure, smoking cessation, • There is no specific treatment; management is largely supportive including
and co-existing chronic bronchitis should be treated treating co-existing TB or bacterial infections, bronchodilators, oxygen avoidance of asbestos, pulmonary rehabilitation and oxygen therapy;
• Patients may be eligible for compensation in the UK via the Industrial therapy, and in some cases lung transplant smoking cessation is important and patients may benefit from influenza
Injuries Act • Patients are usually eligible for compensation as in CWP and pneumococcal vaccinations
• Monitoring to assess for risk of mesothelioma or lung cancer
• Compensation can be claimed as with CWP

Notes
Interstitial lung disease

44 / Chapter 2 Respiratory: Interstitial lung disease Mind Maps for Medicine \ 45

 SCLC (15%) Symptoms

• Arise from Kulchitsky cells • Cough (80%)

• Rapidly growing and highly malignant (almost always inoperable at presentation • Haemoptysis (70%)
but chemoresponsive) • Dyspnoea (60%)
• SCLCs tend to contain membrane-bound neurosecretory granules that can release • Cigarette smoking (approx. • Chest pain (40%)
Most cases of lung cancer are calcitonin, ADH, ACTH and PTH-related peptide (PTHrP) 90% of lung cancers are • Recurrent or slow-resolving pneumonia
bronchial carcinomas which are caused by smoking) • Anorexia
malignant tumours originating from NSCLC (85%) • COPD • Weight loss
the epithelial cell lining of the lower • Previous malignancy,
respiratory tract. They are divided Squamous cell carcinoma (42%): • Lung cancer is the 3rd most particularly head and neck Signs
primarily into small cell lung • Typically arise in proximal segmental bronchi; most present at obstructive lesions of common cancer in the UK, • Industrial dust diseases:
carcinoma (SCLC) and non-small the bronchus accounting for 13% of all asbestos, chromium, arsenic, Locoregional spread:
cell lung carcinoma (NSCLC) • Local spread is common, distant metastases occur late new cancer cases radon gas • Shoulder/inner arm pain/weakness (brachial plexus
depending on histology. • They can also release PTHrP • Lung cancer is the most common • Family history compression)
cause of cancer death in the UK • Increasing age • Horner syndrome (sympathetic ganglion compression)
Adenocarcinoma (39%): • Hoarseness (recurrent laryngeal nerve compression)
Definition • Arise from mucous cells of bronchial epithelium
• Upper limb oedema, facial congestion and distended neck
• The most common bronchial carcinoma associated with asbestos and more common Epidemiology Risk factors veins (superior vena cava obstruction)
in non-smokers
• Even small resectable lesions carry a risk of early occult metastases (most commonly Distant metastases:
spread to the brain and bones) • Personality change, seizures, headaches, focal neurological
signs (brain metastases)
Large cell carcinoma (8%): • Bone pain, back pain, leg weakness (bone metastases and
spinal cord compression)
• These consist of sheets of large, round to polygonal cells with large nuclei and
prominent nucleoli
• They frequently arise centrally and are poorly differentiated Paraneoplastic syndrome:
• Finger clubbing
• Signs of hypercalcaemia (see Ch5: Hypercalcaemia and
Classification/pathophysiology hyperparathyroidism): due to bone metastases or PTHrP
release (in small cell carcinoma or squamous cell carcinoma)
• Hypertrophic osteoarthropathy

Management Lung cancer Clinical features

Management options are described
in full on the following Notes pages

Bloods
Investigations
FBC May show anaemia, raised platelets
U&Es May show hyponatraemia in ADH- Staging and prognosis
producing tumours Histology
Calcium May be ↑ in PTHrP-secreting
• Bronchoscopy Used to obtain biopsies and washings • The TNM (tumour, node, metastasis) staging system is
tumour, or if there is bone metastasis
for cytology used for staging lung cancer
Albumin May be low
• Pleural effusion analysis (see Ch2: Pleural effusion) • Lung cancer should be staged by a contrast-enhanced
Imaging • Sputum Used for people with suspected lung cancer CT scan of the patient’s chest, liver and adrenal glands
who have centrally placed nodules or masses and and by selected imaging of any symptomatic area
• CXR Often 1st investigation done in who decline/cannot tolerate bronchoscopy/other • Prognosis depends on cancer type and the stage of
patients with suspected lung cancer invasive tests cancer
(see Fig. 1) • Transthoracic fine needle aspiration biopsy (under • Overall, the 10-year survival rate for lung cancer in the
• CT chest The investigation of choice to imaging) To obtain histology for peripheral lesions UK is around 5.5%
investigate suspected lung cancer • SCLC has a much poorer prognosis than NSCLC with
• PET scan Typically done in NSCLC to 65–70% of patients having disseminated or extensive
establish eligibility for curative treatment disease at presentation
Fig. 1 Bronchial carcinoma of the left lung

46 / Chapter 2 Respiratory: Lung cancer Mind Maps for Medicine \ 47

 Management of lung cancer Notes
Lung cancer

General management Mesothelioma

• Smoking cessation Definition
• Access to multidisciplinary team
• There is currently no national screening but NHS England is looking at using Mesothelioma is malignancy of mesothelial cells which usually occurs in the
low-dose CT scans as a possible screening test for lung cancer for those of pleura. It can also occur in other sites, including the peritoneum, pericardium
a certain age and who smoke or used to smoke and testes.

NSCLC Epidemiology and Risk factors

• Surgery The treatment of choice for patients with stage I or II disease; • Malignant mesothelioma is three times more common in men than
lobar resection is the procedure of choice in women
• All patients undergoing surgical resection should have hilar and mediastinal • More than 2600 people are diagnosed with the condition each year in the UK
lymph node sampling to provide accurate pathological staging • Almost half of cases of mesothelioma are diagnosed in people aged ≥75
• Radiotherapy Should be offered to all patients with stage I–III NSCLC • Occupational exposure to asbestos accounts for more than 80% of cases;
who are not suitable for surgery there may be a time lag of 20–40 years between exposure and development
• Chemotherapy Should be offered to patients with stage III or IV NSCLC of tumour
and good performance status, to improve survival, disease control and • Crocidolite (blue) asbestos is the most dangerous form
quality of life
• Tyrosine kinase inhibitors e.g. Afatinib, erlotinib and gefitinib are Clinical features
indicated for metastatic NSCLC in individuals with an epidermal growth
factor receptor mutation • Shortness of breath (progressive)
• Chest pain
SCLC • Fatigue, fever and sweats may occur
• Finger clubbing
• Surgery is often not possible (as SCLC metastasises early) • Signs of pleural effusion (see Ch 2: Pleural effusion)
• Chemotherapy Usually 1st line; SCLC is much more chemotherapy • Palpable chest wall mass
sensitive than NSCLC • Signs of metastases: lymphadenopathy, hepatomegaly, bone pain, bone
• Multi-drug treatments are often used tenderness, abdominal pain and gastrointestinal obstruction
• Radiotherapy is an option if there is good response to initial chemotherapy
Investigations
Supportive palliative treatment
• CXR and CT scan may show pleural effusion, lobulated or nodular pleural
• Palliative care team For patients with advanced disease thickening, a pleural mass and rib destruction
• Radiotherapy Used for bronchial obstruction, cough, chest pain, • MRI/PET scan may provide further detail
haemoptysis, superior vena cava (SVC) obstruction, bone and brain • Pleural fluid: straw coloured or bloodstained; cytological analysis may be
metastases, and spinal cord compression useful for diagnosis
• Debulking surgery Considered in bronchial obstruction or for haemoptysis • Pleural biopsy: ultrasound or CT-guided percutaneous biopsy to confirm
• Stent insertion An option for bronchial obstruction and in SVC obstruction diagnosis
• Opiates e.g. Morphine can be used for breathlessness and cough in • Mediastinoscopy and video-assisted thoracoscopy may be useful in
addition to pain determining the stage
• Aspiration or drainage ± pleurodesis Can provide symptomatic relief
from pleural effusions Management
• Bisphosphonates should be considered for bone metastases
Management options are limited:
• Surgery Curative surgery may be possible only in stage I; extrapleural
pneumonectomy may lengthen time to recurrence
• Chemotherapy Palliative chemotherapy has been shown to improve
survival of patients with unresectable mesothelioma
• Radiotherapy May be given in an adjuvant setting after surgery or
chemosurgery although there is little good evidence to support this practice
• Patients are often eligible for compensation in the UK under the
UK Industrial Injuries Act

Staging and prognosis

• The TNM staging is used for staging mesothelioma

• The prognosis remains poor and is typically around 1 year

48 / Chapter 2 Respiratory: Lung cancer notes Mind Maps for Medicine \ 49

 Notes
• Obstructive sleep apnoea (OSA) • Male sex (male to female ratio is 2–3:1) Obstructive sleep apnoea
syndrome is a clinical condition • Irregular breathing results from the upper airway collapsing during sleep • Obesity
in which there is intermittent and • This occurs during sleep as the muscles that hold the airway open are • Large neck circumference
repeated upper airway collapse during hypotonic • Family history of OSA
sleep; this results in irregular breathing • Collapse of the airways can be partial (hypopnoea) or complete • Smoking
at night and excessive sleepiness (apnoea), leading to a temporary arousal from sleep which allows • Alcohol intake before bed
during the day restoration of normal airway muscular tone • Sleeping supine
• Complete apnoea is defined as a • These cycles can occur hundreds of times throughout the night but they • Hypothyroidism
10-sec pause in breathing activity can be so brief that the patient is unaware of them • Acromegaly
• Partial apnoea, also known as • This disturbance to normal sleep pattern leads to reduced sleep quality, • In children: obesity, adenotonsillar
hypopnoea, is characterised by a excessive daytime sleepiness and reduced concentration and alertness hypertrophy and congenital conditions
10-sec period in which ventilation is as a result e.g. Down syndrome, craniofacial
reduced by at least 50% • There are several contributing factors to this (see Risk factors) abnormalities, and Prader–Willi syndrome

Definition Pathophysiology Risk factors

Obstructive sleep apnoea

Clinical features

Symptoms
Complications Management Diagnosis
• Loud snoring
• Daytime somnolence
• Pulmonary hypertension • Behavioural interventions Weight loss, smoking • Simple studies, e.g. pulse oximetry, • Poor sleeping quality
• Type 2 respiratory failure cessation, avoid alcohol during evenings, sleep on side video recordings, may be all that are • Morning headache
• Hypertension • CPAP Still recognised as the gold standard treatment; required • Decreased libido
• Myocardial infarction nasal CPAP (nCPAP) is highly effective in controlling • The Epworth Sleepiness Scale is • Decreased cognitive performance
• Stroke symptoms, improving quality of life and reducing the a simple tool to help discriminate • Irritability/personality change
• Road traffic accidents clinical sequelae of sleep apnoea between OSA and simple snoring
• Bilevel positive airway pressure (BIPAP) Provides • Polysomnography (PSG) is Signs
two different levels of pressure and is an alternative in the traditional gold standard
patients intolerant to CPAP and also in patients with investigation; it usually involves • Obesity
associated hypoventilation or COPD an electroencephalogram (EEG), • Fat deposition anterolateral to the upper airway
• Mandibular advancement devices – increase airway two electro-oculograms (EOGs) to may signify obstruction
diameter with soft tissue displacement by mandibular measure horizontal and vertical eye • Large neck circumference
protrusion movements and an electromyogram • Certain craniofacial or pharyngeal abnormalities
• Pharmacological Role is limited; modafinil may afford to monitor muscle movement (during e.g. retrognathia, micrognathia, enlarged tonsils,
some benefit in some patients sleep): at the end of the investigation, macroglossia, thickening or lengthening of the
• Surgery Removal of markedly enlarged tonsils and the number of apnoea/hypopnoea soft palate or uvula
correction of facial abnormalities episodes is quoted as the apnoea/ • Nasal polyps, rhinitis or any deformity of the nose
hypopnoea index (AHI)
• Arterial blood gases may show
type 2 respiratory failure

50 / Chapter 2 Respiratory: Obstructive sleep apnoea Mind Maps for Medicine \ 51

 Transudates Exudates Symptoms
Normally, the pleural space contains a small physiological
amount of pleural fluid. Pleural effusion occurs when • Heart failure • Infection Empyema, pneumonia, • May be asymptomatic
there is discrepancy between the formation and • Hypoalbuminaemia: cirrhosis, paraneoplastic effusion, TB • Shortness of breath
resorption of pleural fluid. The fluid may be either nephrotic syndrome, malabsorption • Malignancy Lung carcinoma, lymphoma, • Pleuritic chest pain
Pleural effusion is the presence transudative or exudative: • Constrictive pericarditis leukaemia, mesothelioma, lung metastases • Cough (non-productive)
of excessive fluid between the • Transudate Results from abnormal accumulation • Ascites • Inflammatory ARDS, pancreatitis,
visceral and parietal pleura of pleural fluid due to high capillary and interstitial • Hypothyroidism sarcoidosis, radiation Signs
(pleural space). It can comprise a hydrostatic pressures (e.g. in heart failure) or • End-stage kidney disease • Connective tissue disease Churg–
variety of liquids including blood abnormally decreased capillary oncotic pressure • Meigs syndrome (ovarian fibroma Strauss disease, SLE, rheumatoid arthritis, Clinical features •
•
↓Chest expansion on affected side(s)
Stony dull on percussion
(haemothorax), pus (empyema) (e.g. in nephrotic syndrome) producing right-sided pleural effusion) granulomatosis with polyangiitis
and chyle (chylothorax) in the • Exudate Results from inflammatory and malignant • Peritoneal dialysis • Pulmonary embolus With infarction • ↓Breath sounds on affected side(s)
pleural space. Both blood and processes which adjust the permeability of the local • Superior vena cava obstruction • Drugs Methotrexate, amiodarone, • Reduced vocal and tactile fremitus on
air in the pleural space is called capillary and pleural membrane or by lymphatic nitrofurantoin, beta blockers affected side(s)
haemopneumothorax. blockage • Mediastinal shift to opposite side of
Causes effusion (large effusions)
• Signs of underlying cause e.g.
malignancy, hypothyroidism, cardiac
Definition Pathophysiology failure etc.

Pleural effusion Investigations

Bloods Imaging Pleural aspiration and analysis

• FBC WCC raised in infection/ • CXR Loss of costophrenic angle, homogenous white shadow with a concave- • Gross appearance Turbid/yellow
inflammation upwards upper border (meniscal sign), tracheal deviation away from affected (empyema, parapneumonic effusion),
• U&Es Impaired in renal disease side (may occur in massive pleural effusions) (see Fig. 1) haemorrhagic (trauma, malignancy, pulmonary
Management • CRP Raised in infection, inflammation • US Used for determining pleural effusion and guiding diagnostic or therapeutic infarction)
and malignancy aspiration • Microbiology:
• LFTs To rule out liver disease • CT chest To provide more detailed images and help to identify underlying causes • White cells Raised in empyema and exudates;
• BNP/NT-pro BNP To help rule out e.g. malignancy neutrophils raised in parapneumonic effusion,
Acute Long-term
heart failure • Transthoracic ECHO To determine if there is heart failure pulmonary embolism (PE) and abdominal
• Treat underlying cause: e.g. infection • Pleurodesis: chemical pleurodesis • Serum protein* • Pleural biopsy Obtained by CT-guided, blind or video-assisted thoracoscopic diseases; lymphocytes raised in malignancy,
with antibiotics, and diuretics for heart with tetracycline, bleomycin or talc for • Serum LDH* approach; provides tissue diagnosis (TB smear, culture and histology) TB, PE
failure recurrent effusions • Red cells Raised in malignancy, trauma,
Urine *Light’s criteria: helps to determine parapneumonic effusion, PE
• Therapeutic pleural aspiration or • Pleurectomy: for exceptional cases exudate vs transudate
intercostal drainage: if effusion e.g. in mesothelioma and in patients • Culture To identify causative organism in
• Dipstick Protein-positive in nephrotic Light’s criteria are used when it is unclear infection
is symptomatic or large; it is best in good general condition where syndrome
removed slowly pleurodesis has failed whether the pleural effusion is a transudate • Biochemistry:
• Urine protein: creatinine ratio or an exudate. It uses protein and LDH in • Protein <25g/L = transudate,
Raised in nephrotic syndrome the serum and in the pleural fluid. A pleural >35g/L = exudate
effusion is likely exudative if at least one of • Glucose <3.3mmol/L: empyema, malignancy,
the following exists: TB, RA
• pH <7.2: empyema, malignancy, TB
Pleural fluid protein divided by serum • ↑LDH* Any exudative cause
protein >0.5 or • ↑Amylase Pancreatitis, carcinoma, bacterial
Pleural fluid LDH divided by serum pneumonia
LDH >0.6 or • Cytology To help determine whether there is
Pleural fluid LDH more than two-thirds malignancy
Fig. 1 Chest X-ray showing a moderate right the upper limits of normal serum LDH • Immunology RA, ANA, complement levels
pleural effusion

52 / Chapter 2 Respiratory: Pleural effusion Mind Maps for Medicine \ 53

 Community-acquired pneumonia (CAP) Pneumonia in the immunocompromised

Typical: • For example in AIDS, lymphomas, leukaemias, patients

• Streptococcus pneumoniae (80%) taking immunosuppressive drugs
• Haemophilus influenzae • Pneumocystis jirovecii (common opportunistic
An acute lower respiratory tract • Moraxella catarrhalis infection in AIDS)
infection of the lung parenchyma • Staphylococcus aureus • Adenovirus
• CMV
associated with radiographic • Viruses e.g. influenza A, coronavirus (COVID-19) Symptoms
changes. • Herpes simplex virus
• Mycobacterium tuberculosis • Shortness of breath
Atypical:
• Purulent cough (typically green or
• Chlamydia pneumoniae Aspiration pneumonia
Definition • Legionella pneumophila (typically fresh water and man-made water systems)
rust-coloured sputum)
• Haemoptysis
• Mycoplasma pneumoniae • ↑Risk with impairment of swallowing from neurological
abnormalities (e.g. stroke, dementia, Parkinson’s disease), • Fever ± rigors
Hospital-acquired pneumonia (HAP) ↓consciousness, oesophageal disease or iatrogenic • Chest pain (pleuritic)
intervention (e.g. NG tube, bronchoscopy) • Systemic: malaise, anorexia, fatigue
• At least 48–72 h after being admitted • Oropharyngeal anaerobes
• Staphylococcus aureus Signs
• Gram-negative enterobacteria Classification • Look – Cyanosis, ↑RR, confusion
• Klebsiella pneumoniae
• Feel – Fever, tachycardia, dullness on
• Pseudomonas spp.
percussion, ↑tactile fremitus, ↓chest
expansion
• Listen – Bronchial breathing, ↓air entry,
↑vocal resonance, pleural rub

Pneumonia Clinical features

Management
Investigations Severity (CURB 65)

Acute

• Oxygen Keep O2 sats ≥96% (88–92% in those

Complications • Bloods:
• FBC: typically ↑WCC
• Confusion: Abbreviated mental test
score (AMTS) ≤8
with COPD) (SLAPPED HER) • CRP: typically raised • Urea: >7mmol/L
• IV fluids As per SEPSIS 6 if septic or if patient • LFTs: may be deranged in • Respiratory rate: ≥30/min
is dehydrated legionella and sepsis • Blood pressure: systolic <90mmHg or
• Septic shock • U&Es: assessing severity (urea), diastolic ≤60mmHg
• Antibiotics Local microbiology guidelines should
• Lung abscess ↓Na+ in legionella pneumonia • 65 years or above
be followed but examples include amoxicillin for
• Acute kidney injury, Acute respiratory • Serology: for mycoplasma (Each of the above is worth 1 point:
uncomplicated CAP (doxycycline or clarithromycin
distress syndrome • Blood culture 0–1 outpatient care, 1–2 admission,
if penicillin allergic), clarithromycin for atypical
• Pneumothorax • Sputum Gram stain, culture and ≥3 consider ITU treatment)
organisms, co-amoxiclav for HAP (<5 days admission);
• Post-infective bronchiectasis sensitivity
≥5 days after admission: tazocin or a broad-
• Pleural Effusion • CXR Lobar/multi-lobar infiltrates,
spectrum cephalosporin or a quinolone; amoxicillin
• Deep vein thrombosis air bronchogram, pleural effusion,
and metronidazole or co-amoxiclav for aspiration
• Hypotension consolidation (see Fig. 1) – repeat
pneumonia
• Empyema 6 weeks after treatment to ensure
• Analgesia and antipyretics e.g. NSAIDs, paracetamol
• Respiratory failure resolution
• Nebulised saline May aid expectoration
• In severe cases, patients may require non-invasive • Urine For pneumococcal or
ventilation, or intubation and ventilation in ITU legionella antigen
• ABG Type 1 respiratory failure
Prevention Fig. 1 Chest X-ray of a patient with CAP showing left
(↓PO2 with ↔ or ↓PCO2)
lower lobe consolidation
• Influenza and pneumococcal vaccination
• Smoking cessation

54 / Chapter 2 Respiratory: Pneumonia Mind Maps for Medicine \ 55

 Spontaneous pneumothorax • Interstitial lung disease e.g. Sarcoidosis, • The pathophysiology of pneumothorax depends on the underlying
idiopathic pulmonary fibrosis cause Notes
Pneumothorax
Primary: • Connective tissue disease e.g. RA, ankylosing • Primary spontaneous pneumothorax (PSP) occurs in the absence
spondylitis, Marfan syndrome, Ehlers–Danlos of known lung disease but in most patients occurs from the rupture
Occurs in the absence of known
syndrome of blebs and bullae; it typically occurs in tall, young people without
lung disease; risk factors include:
parenchymal lung disease and is thought to be related to increased
• Tall, thin stature Non-spontaneous pneumothorax shear forces in the apex
• Smoking
• Secondary spontaneous pneumothorax (SSP) occurs in the
• Marfan syndrome • Traumatic pneumothorax Follows a penetrating presence of lung disease, most commonly COPD
• Pregnancy chest trauma such as a stab wound, gunshot • Tension pneumothorax typically occurs in blunt traumatic injuries
• Familial pneumothorax injury or a fractured rib e.g. a stab wound; air cannot escape on expiration due to a one-way
A pneumothorax is a life-threatening • Iatrogenic pneumothorax A complication valve mechanism; this results in mediastinal shift and significant lung
condition that refers to a collection Secondary: of medical or surgical procedures e.g. central collapse
of air in the pleural cavity (between line placement, lung biopsy and percutaneous
• Airway disease COPD, asthma,
the lung and the chest wall) resulting liver biopsy
cystic fibrosis
in collapse of the lung on the
• Infection Pneumonia, TB • Catamenial pneumothorax Refers to Pathophysiology
affected side. pneumothorax at the time of menstruation;
• Malignancy Lung cancer
due to thoracic endometriosis leading to
necrotic holes in the diaphragm
Definition
Symptoms
Causes
• Acute SOB
• Acute pleuritic chest pain
• Sudden deterioration in patient with asthma or COPD

Signs

Pneumothorax •
•
↓Expansion on affected side
Hyper-resonance on affected side
• ↓Breath sounds on affected side
• Tracheal deviation to unaffected side (tension
pneumothorax)
• Hypotension (tension pneumothorax)
• Distended neck veins (tension pneumothorax)

Management Clinical features

Complications
Immediate (see Notes for algorithm)
• Further pneumothorax
• High-flow oxygen
Investigations
• Respiratory failure
• Cardiac arrest • Tension pneumothorax requires immediate aspiration in the 2nd intercostal
space mid-clavicular line on the suspected side of pneumothorax (do not delay • CXR Pleural line, reduced vascular markings on affected side (see Fig. 1)
by obtaining a CXR) and deviation of trachea away from affected side in tension pneumothorax
• Observation for patients with a small PSP without breathlessness (see Fig. 2)
• Needle aspiration For PSP (any size) and small SSP in patients <50 years • ABG Type 1 respiratory failure
• Chest drain Indications include: in any ventilated patient, tension pneumothorax • US chest Main value is in management of supine trauma patients
after initial needle relief, persistent or recurrent pneumothorax after simple • CT chest Recommended for uncertain or complex cases
aspiration, large SSP in patients >50 years

Long-term

• Pleurodesis If there has been recurrence or if high risk; involves prevention of

further pneumothorax by obliterating the pleural space; surgical options are more
effective but medical pleurodesis using talc and bleomycin may be appropriate for
patients who are either unwilling or unable to undergo surgery
• Surgery For more difficult cases referral to thoracic surgeons may be considered;
open thoracotomy or video-assisted thoracoscopic surgery (VATS) with
pleurectomy and pleural abrasion; indications include bilateral pneumothoraces,
failure of intercostal drainage, 2 or more previous pneumothoraces on same side
or history of pneumothorax on other side Fig. 1 Left-sided pneumothorax Fig. 2 Right-sided tension pneumothorax
• Smoking cessation Reduces risk of a first pneumothorax and that of a recurrence

56 / Chapter 2 Respiratory: Pneumothorax Mind Maps for Medicine \ 57

 Immediate management of spontaneous pneumothorax Notes
Pneumothorax

Spontaneous pneumothorax
(if bilateral/haemodynamically
unstable proceed to chest drain)

Age >50 and

significant
smoking history,
Primary NO YES Secondary
evidence of
pneumothorax pneumothorax
underlying lung
disease on exam
or CXR?

Size >2cm Aspirate 16–18G Size >2cm

YES YES
and/or cannula and/or
breathless? Aspirate <2.5L breathless?

NO NO

Success?
Aspirate 16–18G
(<2cm NO YES
cannula Size 1–2cm
and breathing
Aspirate <2.5L
improved)

NO
YES

Success?
NO
(size now
<1cm)

YES

Admit, high-flow
Consider discharge
• Chest drain O2 (unless O2
and review in outpatient
• Admit sensitive);
department in 2–4 weeks
observe for 24h

Management of spontaneous pneumothorax: British Thoracic Society pleural disease guideline 2010.

58 / Chapter 2 Respiratory: Pneumothorax notes Mind Maps for Medicine \ 59

 • Deep vein thrombosis (DVT) The most • DVT Symptoms Two-level Wells score (see Fig. 1)
common source of pulmonary emboli is • Previous DVT/PE
a DVT in the lower limbs • Surgery • Dyspnoea • Clinically suspected DVT = 3.0 • Immobilisation >3 days or surgery
• Tumours Most commonly prostate and breast • Obstetrics: late pregnancy, • Chest pain: pleuritic chest pain, retrosternal • Alternative diagnosis less likely ≤4 weeks = 1.5
cancers postpartum, caesarean chest pain than PE = 3.0 • History of DVT or PE = 1.0
• Fat From long-bone fractures • Active cancer • Cough and haemoptysis • Tachycardia (HR >100) = 1.5 • Haemoptysis = 1.0
• Amniotic fluid In pregnant women • Immobility • Dizziness or syncope (right heart failure in • Malignancy = 1.0
Pulmonary embolism (PE) is a
• Sepsis For example: tricuspid valve endocarditis • Increasing age severe cases)
condition in which one or more
in people who inject illicit drugs IV • Oestrogen: COCP, HRT
emboli, usually arising from a Calculate two-level Wells score
• Foreign body e.g. During IV drug use, or from • Obesity Signs
thrombus (blood clot) formed in the
broken catheters, guide wires, vena cava filters etc. • Significant comorbidities:
veins (or, rarely, in the right heart), • Tachypnoea, tachycardia
• Air e.g. heart disease; metabolic,
are lodged in and obstruct the • Hypoxia
(This mind map focuses on thrombotic PE) endocrine, neurological disability <4 >4
pulmonary arterial system. • Pyrexia
• Elevated jugular venous pressure
Definition Sources of emboli Risk factors • Gallop heart rhythm, a widely split second
heart sound, tricuspid regurgitant murmur
Intermediate/
• Pleural rub Low probability
high probability
• Systemic hypotension and cardiogenic
shock (massive PE)

Raised
Clinical features Do d-dimer Start LMWH
Normal
Pulmonary embolism
Seek alternative cause CTPA/VQ scan

Fig. 1 Suggested diagnostic pathway of PE

Calculating risk

Management
Investigations
• ECHO May show signs of right ventricular strain or right ventricular
• Bloods Routine bloods to exclude other causes; d-dimers ↑sensitivity hypokinesis
but ↓specificity; troponin and BNP levels may also be elevated if there • CT pulmonary angiogram (CTPA) Method of choice for imaging the
Acute Long-term is heart strain due to the PE pulmonary vasculature to identify PE (see Fig. 3)
• ECG Any of: normal, sinus tachycardia (most common finding), • Ventilation-perfusion (VQ) scan May be used initially if appropriate
• Oxygen • Warfarin or a direct oral anticoagulant (DOAC) should ideally AF, non-specific ST or T-wave abnormalities, right ventricular strain facilities exist, the CXR is normal, and there is no significant symptomatic
• Heparin: anticoagulate with SC low molecular be commenced within 24h of diagnosis pattern V1–3, right axis deviation, right bundle branch block (RBBB) concurrent cardiopulmonary disease
weight heparin (LMWH) (e.g. tinzaparin 175U/kg • Examples of DOACs include rivaroxaban, apixaban and or ‘S1Q3T3’ pattern (see Fig. 2) • Pulmonary angiography The gold standard for diagnosis but is invasive
or enoxaparin 1.5U/kg) or IV heparin infusion dabigatran; once started, heparin can be discontinued • CXR Usually normal; may show ↓vascular markings, small effusion with significant complication rates compared with above investigations
• Thrombolysis: e.g. alteplase, consider if • Heparin should be continued with warfarin however for 5 days or wedge-shaped area of infarction; mainly useful to exclude other
critically ill or massive PE; 1st-line treatment or until the INR is in the therapeutic range (INR 2–3) chest disease
for massive PE where there is circulatory failure • In some cases, e.g. in a patient with active cancer, LMWH should • ABG May show hypoxia/type 1 respiratory failure
(e.g. hypotension); may be considered also if there be used long term instead of warfarin or DOACs
is evidence of right heart strain (on CTPA or ECHO); • The length of treatment depends on whether the PE is provoked,
the Pulmonary Embolism Severity Index (PESI) unprovoked or recurrent:
score can be used to predict the outcome of PE • Provoked PE: warfarin or DOAC for 3 months; at 3 months
and need for thrombolysis clinicians should assess risks and benefits of extending
treatment
• Unprovoked PEs: warfarin or DOAC for 6 months and rule
out underlying cancer
• Recurrent PEs: lifelong warfarin or DOAC
• Patients with active cancer: LMWH for 6 months

Fig. 2 ‘S1Q3T3’ pattern on ECG Fig. 3 CTPA showing saddle embolus sitting across main pulmonary arteries

60 / Chapter 2 Respiratory: Pulmonary embolism Mind Maps for Medicine \ 61

 Type 1 respiratory failure Type 1 respiratory failure
Notes
Acute respiratoryRespiratory
distress syndrome
failure
Defined as hypoxia (PaO2 <8kPa) with a • Pneumonia
normal or low PaCO2. It is caused primarily • Pulmonary oedema
by ventilation/perfusion mismatch. • Pulmonary embolism
• Asthma
Type 2 respiratory failure • Pulmonary fibrosis
• Pneumothorax
Respiratory failure occurs when gas Defined as hypoxia (PaO2 <8kPa) with
• Bronchiectasis
exchange is inadequate, resulting in hypercapnia (PaCO2 >6kPa). This is caused
• Acute respiratory distress syndrome
hypoxia. It is defined as a PaO2 <8kPa by alveolar hypoventilation with or without
and classified as either type 1 or ventilation/perfusion mismatch. Type 2 respiratory failure
type 2, based on whether there is a
high CO2 level. • Obstructive pulmonary disease Asthma (severe),
COPD, obstructive sleep apnoea
• Reduced respiratory drive Sedative drugs,
CNS tumour or trauma
Definition Classification • Neuromuscular disease Cervical cord lesion,
diaphragmatic paralysis, poliomyelitis, myasthenia
gravis, Guillain–Barré syndrome
• Thoracic wall disease Flail chest, kyphosis, scoliosis

Causes

Respiratory failure

Management Investigations Clinical features

Type 1 respiratory failure • Bloods: FBC, U&Es, CRP Hypoxia Hypercapnia

• ABG
• Treat the underlying cause • Peak expiratory flow rate • Dyspnoea • Headache
• Give O2 via facemask to correct hypoxia • Chest radiology: CXR, CT • Restlessness • Peripheral
• Assisted ventilation if PaO2 <8kPa despite 60% O2 • Microbiology: sputum and • Agitation vasodilatation
60%
15
LM
N blood cultures • Confusion • Tachycardia
Type 2 respiratory failure • Spirometry • Central cyanosis • Bounding pulse
• ECG • Long-standing • Tremor/flap
• Treat the underlying cause polycythaemia, • Papilloedema
• Controlled oxygen therapy via venturi mask; start at 24% O2; 24% 35% 31% 28% 40% pulmonary • Confusion
O2 therapy should be given with care but hypoxia should not 3 LMN 5 LMN 4 LMN 4 LMN 10 LMN
hypertension, cor • Drowsiness
be left untreated pulmonale • Coma
• Recheck the arterial blood gas after approx. 20min;
if PaCO2 is steady or lower, increase O2 concentration Interchangeable venturi valves
in stages (see Fig. 1)
• If PaCO2 has risen above 1.5kPa and the patient is still hypoxic, Fig. 1 Venturi masks are used to deliver
consider a respiratory stimulant or assisted ventilation controlled percentages of O2 in patients at
(e.g. NIPPV, non-invasive positive pressure ventilation) risk of type 2 respiratory failure
• If improvement with NIPPV, continue and adjust settings
accordingly
• If this fails consider intubation and ventilation if appropriate

62 / Chapter 2 Respiratory: Respiratory failure Mind Maps for Medicine \ 63

 • It is thought that one or more unidentified antigen(s) triggers activation of Symptoms Syndromes associated with sarcoidosis
T-helper cells followed by the development of non-caseating granulomata
in genetically susceptible individuals • Constitutional symptoms Fever, night sweats, malaise, weight loss • Löfgren syndrome An acute form of the disease characterised by bilateral
• The granulomas consist of macrophages, epithelioid cells and • Lymph nodes Commonly picked up on CXR but may be symptomatic hilar lymphadenopathy, erythema nodosum, fever and polyarthralgia; it
A multisystem chronic inflammatory multinucleated giant cells surrounded by lymphocytes, monocytes, mast and affect the axillary, cervical and inguinal nodes usually carries an excellent prognosis
condition characterised by the cells and fibroblasts • Lungs Cough (usually dry), breathlessness, wheeze, fine crackles • Mikulicz syndrome Enlargement of the parotid and lacrimal glands due to
formation of non-caseating epithelioid • The accumulation of T lymphocytes, mononuclear phagocytic cells and • Skin Erythema nodosum (Fig. 1), lupus pernio, infiltration of scars by sarcoidosis, TB or lymphoma
granulomata at various sites in the body: non-caseating granulomas occurs in involved organs; these granulomas granulomas • Heerfordt syndrome Parotid enlargement, fever and uveitis secondary to
most commonly the lungs, lymph nodes, may resolve spontaneously or lead to secondary fibrosis and permanent • Eyes Uveitis (anterior and posterior), dry sarcoidosis
eyes and skin. In approximately 50% of organ damage eyes, glaucoma
cases the disease is detected incidentally • Sarcoidosis involves the lungs in >90% of cases and commonly the • Heart Arrhythmia, cardiomyopathy, heart
on routine CXR in an asymptomatic lymphoreticular system, skin, eyes, muscles and joints; less commonly other failure, conduction defects
individual. organs, including the heart, kidneys, brain and the peripheral nervous system • Neurological Meningeal inflammation,
seizures, mass lesions, neuropathy,
hypothalamic pituitary infiltration Clinical features
Definition Pathophysiology • Hypercalcaemia Nephrolithiasis, Fig. 1 Erythema nodosum
neuropsychiatric disturbance, abdominal
pain, bone pain
• Liver Commonly involved but rarely clinically significant, hepatitis,
hepatosplenomegaly

Sarcoidosis
Staging

There are 5 stages of sarcoidosis according to CXR findings (see Fig. 2)

Prognosis • Stage 0 Normal CXR findings
Management Investigations • Stage I Bihilar lymphadenopathy

Poor prognostic factors

• Stage II Bihilar lymphadenopathy + parenchymal infiltrates Epidemiology and risk factors
• Stage III Parenchymal infiltrates alone
Steroids • Bloods FBC (WCC usually raised), CRP/ESR (usually • Stage IV Pulmonary fibrosis
• Insidious onset, symptoms
>6 months raised), calcium (usually raised), LFTs (deranged if
Stage I Stage II
• Absence of erythema nodosum • Oral prednisolone or IV hydrocortisone liver involvement), ACE level raised in approx. 60% of • Age: occurs at all ages, although it usually develops
(lymphadenopathy) (lymphadenopathy and infiltrates)
• Extrapulmonary manifestations • Indication for steroids include: patients with acute disease and decreases in response before the age of 50 years, with the incidence peaking
e.g. lupus pernio, splenomegaly • CXR stage II or III who have to treatment/resolution of the disease at 20–39
• CXR: stage III–IV features moderate to severe or progressive • CXR (see Staging) • Can occur in all ethnic groups but the disease is more
• Black ethnicity symptoms • ECG To check for early signs of rhythm disturbance severe in African–Caribbeans than Caucasians
• Hypercalcaemia due to conducting system disease or effects of • Family history increases risk
• Eye, heart or neuro involvement hypercalcaemia • HLA-B8 antigens and HLA-DRB1 and -DQB1 alleles
• Tuberculin test Typically negative in patients with have been shown to confer susceptibility
DMARDs/biological agents sarcoidosis • Environmental associations: emissions from wood-
• CT chest Used to detect and assess the severity of burning stoves and tree pollen, exposure to inorganic
• Antimetabolites, e.g. methotrexate,
interstitial lung disease particles, insecticides and mouldy environments have
azathioprine, leflunomide and
• Lung function tests Show restrictive defect in severe, been reported; positive associations have also been
mycophenolate, are alternatives
progressive cases made with service in the US Navy, metalworking,
to steroids
• Bronchoalveolar lavage ↑Lymphocytes, ↑CD4:CD8 firefighting and the handling of building supplies;
• TNF alpha inhibitors, e.g. infliximab
ratio bacteria such as mycobacteria have also been linked
and adalimumab, may be prescribed
• Biopsy Transbronchial, lymph node or skin lesion with sarcoidosis
if patients not responding to
biopsies demonstrate non-caseating granulomas
antimetabolites
• FGD PET scan Can be used to assess inflammatory
Surgical activity accurately in patients
• Gallium scan May be used to detect extrapulmonary
• Surgery may be considered with disease and tends to reveal a ‘lambda’ pattern Stage III Stage IV
(infiltrates only) (fibrosis)
severe progressive lung fibrosis,
including lung transplantation (rare) Fig. 2 Stages I–IV of sarcoidosis

64 / Chapter 2 Respiratory: Sarcoidosis Mind Maps for Medicine \ 65

 Chapter 3
03
Gastroenterology

Acute liver failure..........................................................................................................................................................68

Acute pancreatitis........................................................................................................................................................70
Alpha-1 antitrypsin deficiency.............................................................................................................................72
Chronic pancreatitis....................................................................................................................................................74
Cirrhosis..............................................................................................................................................................................76
Coeliac disease...............................................................................................................................................................80
Crohn’s disease...............................................................................................................................................................82
Gastric cancer.................................................................................................................................................................86
Gastro-oesophageal reflux disease...................................................................................................................88
Hereditary haemochromatosis............................................................................................................................90
Irritable bowel syndrome........................................................................................................................................92
Jaundice.............................................................................................................................................................................94
Oesophageal cancer and other causes of dysphagia............................................................................96
Peptic ulcer disease..................................................................................................................................................100
Primary biliary cholangitis....................................................................................................................................102
Primary sclerosing cholangitis...........................................................................................................................104
Ulcerative colitis.........................................................................................................................................................106
Upper gastrointestinal bleed.............................................................................................................................108
Wilson’s disease..........................................................................................................................................................112

Mind Maps for Medicine \ 67

 Bernau’s classification • Infection Viral hepatitis (mainly • Wilson’s disease • Jaundice impaired performance of
hepatitis A or B, it is extremely • Autoimmune hepatitis • Encephalopathy (classification): subtraction
• Fulminant hepatic failure (FHF) Liver failure takes place within uncommon in hepatitis C), • Budd–Chiari syndrome • Grade 0 No personality or • Grade 3 Somnolence to
8 weeks of the onset of the underlying illness leptospirosis, yellow fever, EBV, • Pregnancy related Acute behavioural abnormality semi stupor but responsive
• Subacute FHF or late-onset hepatic failure Occurs when there has CMV, herpes simplex virus, fatty liver of pregnancy, HELLP detected to verbal stimuli, confusion,
Acute liver failure (ALF) is an
been a gap of 8–26 weeks between the onset of illness and liver failure dengue virus syndrome • Grade 1 Lack of awareness, gross disorientation
uncommon but life-threatening
• Drugs Paracetamol overdose • Reye syndrome Rare side effect euphoria or anxiety, • Grade 4 Coma
illness in which rapid deterioration
O’Grady’s classification (most common cause in of aspirin shortened attention span, • Fetor hepaticus
of the liver function results in
developed countries), halothane, • Ischaemic hepatitis e.g. From impaired performance of • Asterixis/hepatic flap
coagulopathy and alteration in the • Hyperacute Liver failure occurring <7 days from disease onset isoniazid, phenytoin, amiodarone, shock or heart failure addition • Constructional apraxia
mental status of a previously healthy • Acute Liver failure occurring 1–4 weeks from disease onset propylthiouracil, Ecstasy, herbal • Indeterminate • Grade 2 Lethargy or apathy, • Hypoglycaemia
individual. • Subacute Liver failure occurring 4–12 weeks from disease onset remedies, amoebic infection minimal disorientation • Bleeding and bruising
• Toxins Amanita phalloides for time or place, subtle • Hepatomegaly
Definition Classification mushroom toxin, carbon Causes personality change, • Ascites
tetrachloride, yellow phosphorus inappropriate behaviour,

Clinical features

Notes
Acute liver failure
Acute liver failure

Management Complications Investigations

Supportive measures and monitoring • Manage infections Broad-spectrum Criteria for paracetamol-induced
antibiotics e.g. IV ceftriaxone • Cerebral oedema • Bloods FBC, ESR, CRP, LFTs,
liver failure
• Should be managed in specialised liver unit or ITU • Manage hypoglycaemia IV dextrose • Hypoglycaemia U&Es, glucose, ANA, AMA, SMA,
• Arterial pH <7.30 (24h after ingestion) • Hypotension immunoglobulins, prothrombin
setting with regular monitoring • Manage ascites Restrict fluid, low-salt diet,
• Patients who have advanced encephalopathy should daily weights, diuretics OR all of the following: • Hepatorenal syndrome time/INR, hepatitis screen, EBV
have airways protected with intubation and NG tube • Manage hypotension Monitor with arterial • INR >6.5 (PT >100sec) • Sepsis and CMV serology, paracetamol
inserted to avoid aspiration and remove any blood line, fluid resuscitation • Creatinine >300µmol/L • Seizures and salicylate levels
from stomach • Manage encephalopathy Avoid sedatives, • Grade III or IV hepatic encephalopathy • Acute respiratory distress • ABG
correct electrolytes, lactulose syndrome • Ceruloplasmin and 24-h urine
Treat underlying cause • Treat seizures Phenytoin copper If suspected Wilson’s
Criteria for non-paracetamol-
• Manage cerebral oedema Patients should disease
• e.g. IV NAC for paracetamol overdose, stop offending induced liver failure
be positioned with the head elevated at 30° • Blood cultures
drugs, steroids for autoimmune hepatitis Prothrombin time >100sec (INR >6.5) • Doppler of hepatic veins If
in ITU; patient should have ICP monitoring;
OR any three of the following (PADDS): suspected Budd–Chiari syndrome
Manage complications treat with IV mannitol and hyperventilation
• Prothrombin time >50sec (INR >3.5) • CXR
Emergency liver transplantation • Age <10 or >40 years • US abdomen
• Treat bleeding/raised INR Vitamin K e.g. 10mg IV, • Drug-induced liver failure
platelets, fresh frozen and red cells as needed • Ascitic tap If ascites present
King’s College Criteria for liver transplant • Duration of jaundice to hepatic
• Treat renal failure Haemofiltration or haemodialysis encephalopathy >7 days
Predicts poor outcome in acute liver failure and
should prompt consideration for transplantation • Serum bilirubin >300µmol/L

68 / Chapter 3 Gastroenterology: Acute liver failure Mind Maps for Medicine \ 69

 • A trigger, e.g. alcohol or any of the causes listed, is Symptoms
thought to activate a common pathway leading to
marked elevation of intracellular calcium leading • Acute upper abdominal pain
to activation of intracellular proteases and release (may radiate to the back)
of exocrine pancreatic enzymes • Gallstones  • Nausea/vomiting
 By far the most common causes
Acute inflammation of the pancreas • This results in acinar cell injury and necrosis which • Ethanol  • Anorexia
causing the release of exocrine promote migration of inflammatory cells • Trauma • Pyrexia
enzymes that cause autodigestion of • This leads to local inflammatory response and • Steroids
the organ. There may be involvement occasionally a systemic inflammatory response, • Mumps (other viruses include Coxsackie B) Signs
of local tissues and distant organs. resulting in single or multi-organ failure • Autoimmune (e.g. polyarteritis nodosa), Ascaris
infection • Fever, hypotension, tachycardia, tachypnoea
• Scorpion venom • Epigastric tenderness, with guarding on
Definition Pathophysiology • Hypertriglyceridaemia, Hyperchylomicronaemia, abdominal examination
Hypercalcaemia, Hypothermia • Decreased bowel sounds
• ERCP • Jaundice
• Drugs e.g. azathioprine, bendroflumethiazide, • Grey Turner’s sign (ecchymosis of the Fig. 1 Cullen’s sign (A) and Grey Turner’s sign (B): signs of
furosemide, mesalazine, steroids, sodium valproate flank) and Cullen’s sign (ecchymoses in the retroperitoneal haemorrhage (a late clinical feature of acute
periumbilical region) (see Fig. 1) pancreatitis)

Causes (GET SMASHED)

Clinical features

Acute pancreatitis Notes

Acute pancreatitis

Complications Management Assessing severity Investigations

Local (the Ps) • Initially nil by mouth and resuscitation Glasgow Imrie criteria (PANCREAS) at 48h
with IV fluids after admission: Bloods Imaging
• Pseudocyst • Oxygen • PaO2 (arterial) <8.0kPa
• Pancreatic abscess • Pain relief e.g. with opioids • Age >55 years • Pancreatic enzymes: serum • AXR (erect) Excludes other
• Pancreatic necrosis • Antibiotics (broad spectrum) • Neutrophilia White blood cells >15×109/L amylase raised (typically ≥3× differentials, e.g. intestinal
• Pancreatic fistulae For treatment of associated cholangitis • Calcium <2.0mmol/L upper limit of normal); lipase obstruction and perforation, and
• Chronic Pancreatitis or other acute infections; if pancreatic • Renal (serum urea) >16mmol/L levels are more sensitive and may show pancreatic calcification
necrosis is suspected, IV antibiotics should • Enzymes LDH >600IU/L more specific • CXR May show elevation of one
Systemic (DR SAM) be given • Albumin <32g/L • FBC, U&Es, glucose and hemidiaphragm, infiltrates ± acute
• Severe cases should be treated in ITU or • Sugar (glucose) >10mmol/L CRP indicate prognosis respiratory distress syndrome or
• Disseminated intravascular a HDU (see Assessing severity) pleural effusions (in severe cases)
coagulation (≥3 points: high risk for severe pancreatitis)
• Nutritional support Oral feeding can • Raised bilirubin and/or serum • CT pancreas (contrast-enhanced)
• Renal failure (acute) Other scoring systems for prognosis include:
commence in people with mild acute aminotransferase (suggest Can identify pancreatic swelling,
• Sepsis Ranson’s Criteria/Acute Physiology and
pancreatitis (in absence of nausea/ gallstones) fluid collection and change
• Acute respiratory distress Chronic Health Evaluation II (APACHE II)
vomiting, or abdominal pain); enteral • Calcium (hypocalcaemia is in density of gland; this has
syndrome as well as measurement of CRP
feeding is otherwise preferable and relatively common and may implications for prognosis and
• Multiple organ dysfunction is possible in the majority of people; indicate prognosis) predicts the need for surgery
parenteral feeding is reserved for people • US abdomen Can show a swollen
in whom enteral nutrition is not possible pancreas, dilated common bile duct
and free peritoneal fluid; it is also
useful for detecting gallstones
• MRI pancreas May reveal acute
abdominal wall oedema which may
be useful in assessing severity

70 / Chapter 3 Gastroenterology: Acute pancreatitis Mind Maps for Medicine \ 71

 • The gene for A1AT is found on • Patients who manifest disease Lungs Liver
chromosome 14 and is inherited usually have PiZZ genotype
in an autosomal recessive fashion • If there is a deficiency of A1AT • Lung disease does not usually present (Not everyone with A1AT deficiency will develop liver
• Alleles are classified by their then elastase can break down until people are in their 30s or 40s, disease)
electrophoretic mobility: M, normal; elastin unchecked; in the lungs with smokers tending to develop
Alpha-1 antitrypsin (A1AT) S, slow; and Z, very slow: this can cause destruction symptoms approx. 10 years earlier than Neonates/children
deficiency is a common inherited • Normal = PiMM of alveolar walls resulting in non-smokers
condition caused by lack of the • Homozygous PiSS (50% normal emphysema • Neonates may present with neonatal jaundice and
• COPD symptoms: dyspnoea, wheezing
protease inhibitor (Pi) A1AT normally A1AT levels) • Some people may develop liver hepatitis
and cough most common (see Ch2:
produced by the liver. It classically • Homozygous PiZZ (10% normal disease due to congestion of • Older children may develop hepatitis, cirrhosis and liver
Chronic obstructive pulmonary disease)
causes emphysema (i.e. COPD) A1AT levels) A1AT in the liver cells causing failure
• There is increased risk of lung cancer
in patients who are young and • The role of A1AT is to protect cells from cell destruction
non-smokers. enzymes such as neutrophil elastase Adults
• May develop hepatitis, fibrosis, cirrhosis and liver failure
• Increased risk of hepatocellular carcinoma (due to above)
Definition Pathophysiology

Clinical features

Alpha-1 antitrypsin deficiency

Notes
Alpha-1 antitrypsin deficiency

Management Investigations Epidemiology

Conservative • Serum A1AT concentrations • A1AT deficiency is one of the

Reduced most common inherited disorders
• Smoking cessation • LFTs May be deranged in liver among Caucasians
• Pulmonary rehabilitation disease • Approx. 1 per 3000–5000
• Spirometry Obstructive picture individuals are affected by A1AT
Pharmacological if emphysema present deficiency
• CXR/CT chest May show • Up to 5% of people diagnosed
• Bronchodilators (see Ch2: Chronic obstructive evidence of emphysema with COPD are thought to have
pulmonary disease) • Liver biopsy A1AT-containing A1AT deficiency
• IV A1AT protein concentrates: particularly globules are seen in hepatocytes
for non-smokers with COPD attributable to
emphysema; treatment is very expensive

Surgical

Lung
• Lung volume reduction surgery
• Lung transplantation

Liver
• Liver failure may require liver transplantation

72 / Chapter 3 Gastroenterology: Alpha-1 antitrypsin deficiency Mind Maps for Medicine \ 73

 • The underlying mechanism is unclear and chemokines, leading to the deposition • Alcohol Approx. 70–80% of cases • Drugs e.g. Thiazide diuretics, • Abdominal pain: classically
• Abnormalities of bicarbonate excretion of extracellular matrix • Idiopathic Approx. 25% of cases azathioprine, tetracyclines, epigastric pain radiating into
caused by functional defects at the level • Alcohol causes proteins to precipitate in • Smoking An independent risk factor valproic acid and DDP-4 inhibitors the back
of the cellular wall, e.g. in cystic fibrosis, the ductular structure of the pancreas; • Congenital Pancreas divisum, annular • Trauma • Nausea and vomiting
or mechanical causes e.g. trauma, can this leads to local pancreatic dilatation pancreas • Obstruction of the pancreatic • Decreased appetite
Chronic pancreatitis is a chronic, result in pancreatic enzyme activation and fibrosis; there may also be direct toxic • Familial Hereditary pancreatitis, duct e.g. Pseudocysts, ductal • Malabsorption with weight
irreversible inflammation and/or • Pancreatic enzyme activation leads to effects of alcohol on the pancreas cystic fibrosis strictures, periampullary tumours, loss, diarrhoea, steatorrhea
fibrosis of the pancreas, typically pancreatic tissue injury and necrosis • Oxidative stress seems to play an important • Metabolic Hypercalcaemia, gallstones (uncommon) and protein deficiency
characterised by severe abdominal • Pancreatic fibrogenesis is a typical role in the pathogenesis of chronic hyperlipidaemia • Autoimmune disease Sjögren • Diabetes mellitus
pain and progressive endocrine and response to injury involving a complex pancreatitis • Iatrogenic e.g. ERCP, abdominal syndrome, IBD and primary biliary (see Ch5: Diabetes mellitus)
exocrine insufficiency. interplay of growth factors, cytokines radiotherapy cirrhosis

Clinical features
Definition Pathophysiology Aetiology

Chronic pancreatitis
Investigations

Bloods Imaging Stool

Serum amylase (usually normal but • AXR 30% show pancreatic calcification in later stages • Faecal elastase (reduced if
may be mildly raised in an acute on (see Fig. 1). malabsorption present)
chronic attack), albumin and clotting • Abdominal US Reveals gallstones, duct dilatation,
Complications Management studies (may be deranged in liver pancreatic morphology
disease), low calcium and B12 (suggests • Abdominal CT
malabsorption), ALP (raised levels 1st-line imaging
Common Conservative measures Surgery suggests biliary tract obstruction modality for people
if gamma-glutamyltransferase is with a history
• Malabsorption • Alcohol cessation • Cholecystectomy Clearance of also raised), raised glucose/HbA1c and symptoms
• Diabetes mellitus • Smoking cessation duct stones – essential if present (in endocrine dysfunction), serum suggestive of chronic
• Chronic pain • Dietitian support • Sphincterotomy Accessory papilla trypsinogen (low) alcohol-related
• Opioid dependency due in patients with pancreas divisum pancreatitis
to chronic pain Pain relief • Percutaneous or surgical • MRCP/ERCP Useful
• Osteoporosis drainage (laparoscopic or open) to look for treatable
• Pseudocyst formation • Simple analgesia initially e.g. paracetamol Pseudocyst or abscess strictures, tumours,
• Pancreatic calcification and NSAIDs • Partial pancreatic resection stones or pseudocysts
• Opiates e.g. tramadol A Whipple’s procedure may be • Endoscopic US
Less common • Coeliac plexus block via a gastric approach under necessary Irregular duct walls,
endoscopic US guidance • Extracorporeal shockwave duct dilatation and Fig. 1 Calcification of pancreas on AXR
• Duodenal/gastric outlet • ERCP may help to reduce pain by dilating lithotripsy For adults with cysts can be detected
obstruction strictures of the pancreatic ducts pancreatic duct obstruction caused
• Biliary obstruction by a dominant stone if surgery is
• Pancreatic cancer Managing pancreatic insufficiency
unsuitable
• Fistulae • Total pancreatectomy A last
• Splenic or portal vein thrombosis • Replacement of pancreatic enzymes can help
malabsorption and also reduce pain e.g. Creon® resort for relief of intractable pain
• Pseudoaneurysm not responding to other methods
• Fat-soluble vitamins (A, D, E, K)
• Treat diabetes mellitus accordingly e.g. insulin

74 / Chapter 3 Gastroenterology: Chronic pancreatitis Mind Maps for Medicine \ 75

 • Liver fibrosis results from chronic insult • Macronodular (often seen in • Alcohol Excess (Compensated cirrhosis may be asymptomatic)

(due to causes listed) resulting in chronic viral hepatitis) depending on • Viral hepatitis Approx. 10% of cases (types B, C and D)  Most common causes
Cirrhosis results from necrosis of liver cells followed abnormal connective tissue production the size of nodules, although a mixed • Non-alcoholic fatty liver disease (NAFLD)  Decompensated clinical features
by fibrosis and nodule formation. This results in and deposition picture is also seen • Genetic: • Autoimmune:
impairment of liver cell function and gross distortion • Cirrhosis is an advanced stage of • The major clinical consequences of • Jaundice
• Haemochromatosis • Primary biliary cholangitis (PBC)
of the liver architecture, leading to complications such liver fibrosis that is accompanied cirrhosis are impaired hepatocyte • Signs of portal hypertension: ascites, caput medusae,
• Alpha-1 antitrypsin deficiency • Primary sclerosing cholangitis
as portal hypertension. by distortion of the hepatic function, increased intrahepatic splenomegaly, variceal haemorrhage
• Wilson’s disease (PSC)
Cirrhosis can be described as: architecture resistance and the development • Signs of hepatic encephalopathy
• Galactosaemia (rare) • Autoimmune hepatitis
• Compensated The liver can still function effectively • Historically, cirrhosis can be divided of hepatocellular carcinoma • Congenital tyrosinaemia • Sarcoidosis Features of chronic liver disease
with few or no clinical symptoms into 2 types histologically: • Multiple circulatory abnormalities • Glycogen storage disease type IV • Hepatic venous outflow
• Decompensated The liver is damaged to the point • Micronodular (often caused in cirrhosis are closely linked to the • Cystic fibrosis obstruction: • Nails: leuconychia, Terry’s nails, clubbing
that it cannot function adequately with evident by alcohol excess or biliary hepatic vascular alterations resulting • Hepatic veno-occlusive disease • Palmar erythema
clinical complications (see Clinical features) tract disease) or in portal hypertension • Budd–Chiari syndrome • Dupuytren’s contracture
• Congestive hepatopathy (from • Spider naevi (see Fig. 1)
constrictive pericarditis) • Xanthelasma
Definition Pathophysiology Causes • Drugs e.g. Methotrexate, • Hepatomegaly
amiodarone, methyldopa • Gynaecomastia (see Fig. 2), atrophic testes, loss of body hair
• Parotid enlargement

Cirrhosis
Fig. 1 Spider naevi

Complications Management Investigations

• Portal hypertension Manage underlying cause Manage complications of cirrhosis • Bloods:

• Ascites and slowing progression • FBC (occult bleeding may cause anaemia, macrocytic anaemia in alcohol
• Screening and management of oesophageal misuse, ↓WCC and platelets may indicate hypersplenism)
• Portosystemic encephalopathy
• Alcohol abstinence varices (see Ch3: Upper gastrointestinal bleed) • Biochemistry: LFTs (may show ↑bilirubin, ALT, AST, gamma-GT), clotting Fig. 2 Gynaecomastia
• Acute kidney injury (hepatorenal
• Ensure adequate nutrition, including calorie • Cholestyramine to help with pruritus and albumin (↓albumin and ↑INR if synthetic function affected), U&Es
syndrome)
and protein intake • Manage hepatic encephalopathy appropriately (may show ↓Na+ and renal function deranged in hepatorenal syndrome),
• Hepatopulmonary syndrome
• HCC
• Treat haemochromatosis accordingly e.g. with oral lactulose glucose (may be deranged) Clinical features
e.g. venesection (see Ch3: Haemochromatosis) • Management of ascites (see Notes) • Specific tests for underlying cause: serum alpha-fetoprotein (↓ in
• Bacteraemia, infection
• Treat viral hepatitis accordingly • Screening and management of osteoporosis alpha-1 antitrypsin deficiency), ceruloplasmin (if suspected Wilson’s
• Malnutrition
(see Ch8: Hepatitis sections) (see Ch7: Osteoporosis) disease); iron studies and HFE gene mutation analysis (in suspected
• Osteoporosis
• Steroids for autoimmune chronic active haemochromatosis); viral hepatitis screen (if suspected viral hepatitis
hepatitis Monitoring and screening cause); enhanced liver fibrosis (ELF) test in people with NAFLD to test
• Avoid hepatotoxic drugs for hepatocellular carcinoma (HCC) for advanced liver fibrosis; autoantibodies (ANA, AMA, SMA) to rule out
autoimmune hepatitis and PBC
US liver ± alpha-fetoprotein every 6 months as
• US abdomen To detect cirrhosis and hepatocellular carcinoma (HCC) and
surveillance for HCC
also to assess for splenomegaly and ascites
Liver transplantation • Liver duplex To assess for hepatic venous outflow obstruction
• CT/MRI liver To detect complications of cirrhosis, such as splenomegaly,
The only definitive treatment for cirrhosis ascites or HCC
• Ascitic tap For ascites to identify cause and rule out infection
• Liver biopsy Confirms diagnosis

76 / Chapter 3 Gastroenterology: Cirrhosis Mind Maps for Medicine \ 77

 Cirrhosis notes Notes
Cirrhosis

Assessing severity of cirrhosis: Clinical features

Child–Pugh score (ABCDE) • Abdominal swelling/fullness
Child–Pugh score is used to assess the prognosis of chronic liver disease, • Shifting dullness
mainly cirrhosis: • Pleural effusion and peripheral oedema may also be present
• Albumin (serum, mmol/L): <34 (1 point), 34–50 (2 points), >50 • Other signs of underlying cause may also be present.
(3 points)
• Bilirubin (total, g/L): <34 (1 point), 34–50 (2 points), >50 (3 points) Diagnosis
• Coagulation INR: <1.7 (1 point), 1.7–2.3 (2 points), >2.3 (3 points)
• Bloods LFTs including serum albumin, specific tests (see above)
• ‘Drum’ abdomen (Ascites): none (1 point), mild (2 points), severe
depending on clinical features
(3 points)
• Ascitic tap:
• Encephalopathy (hepatic): none (1 point), grade I–II (2 points),
• Ascitic albumin to help differentiate between transudate and
grade III–IV or refractory (3 points)
exudate (≥11g/L suggests exudate and <11g/L suggests
Interpretation: transudate)
• Class A (5–6 points) = 1-year survival (100%), 2-year survival (85%) • WCC, Gram stain and culture; neutrophil count >250cells/mm3
• Class B (7–9 points) = 1-year survival (80%), 2-year survival (60%) suggests bacterial (usually spontaneous peritonitis)
• Class C (10–15 points) = 1-year survival (45%), 2-year survival (35%) • Cytology for malignant cells
• Amylase to exclude pancreatitis
Ascites The serum-ascites albumin gradient (SAAG) is a formula which can further
help to determine the cause of ascites:
Definition SAAG = (albumin concentration of serum) – (albumin
concentration of ascitic fluid)
An abnormal accumulation of fluid within the peritoneal cavity. A high gradient (>1.1g/dL) indicates the ascites is due to portal
hypertension with 97% accuracy.
Pathophysiology
Management
• Ascitic fluid can accumulate as a transudate or an exudate
• Broadly speaking, transudates are a result of increased pressure in the • Treat underlying cause
hepatic portal vein, e.g. due to cirrhosis, while exudates are actively • Bed rest, fluid and salt restriction
secreted fluid due to infection, inflammation or malignancy • Diuretics e.g. Spironolactone (50–100mg/day); furosemide can also
• In cirrhosis, vasodilators are locally released due to portal hypertension; be added
these affect the splanchnic arteries causing reduced arterial blood flow • Paracentesis Used in patients with tense ascites or those who are
and arterial pressures leading to activation of mechanisms including resistant to above treatments; provides rapid symptomatic relief; IV
the renin–angiotensin–aldosterone system, sympathetic nervous infusion of albumin is administered immediately afterwards
system and ADH which results in Na+ and water retention • Transjugular intrahepatic portosystemic shunt (TIPS) Occasionally
• The formation of oedema is also further exacerbated by used for resistant ascites caused by portal hypertension
hypoalbuminaemia • Treat spontaneous bacterial peritonitis accordingly with antibiotics
according to local antimicrobial guidelines after diagnostic aspiration
Causes

Transudate Exudate

Portal hypertension e.g. cirrhosis Cancer: metastasis or primary

carcinomatosis
Hepatic veno-occlusive disease Infection: including peritoneal TB

Budd–Chiari syndrome Nephrotic syndrome

Cardiac failure Lymphatic obstruction

Meigs syndrome Serositis

Constrictive pericarditis

78 / Chapter 3 Gastroenterology: Cirrhosis notes Mind Maps for Medicine \ 79

 • Coeliac disease is strongly associated • Chronic or intermittent
with HLA-DQ2 (95% of patients) and diarrhoea
Coeliac disease is an immune- HLA-DQ8 (80%) • Recurrent abdominal pain,
mediated, inflammatory systemic • The toxin alpha-gliadin is the toxic cramping or distension
disorder provoked by gluten and portion of the gluten which is contained • Failure to thrive (in children)
related prolamins in genetically in wheat, rye and barley • Nausea and vomiting
Normal
susceptible individuals. This can • Gliadin is deaminated by tissue • Fatigue
result in malabsorption of nutrients transglutaminase (TTG); it then interacts • Sudden or unexpected
due to chronic inflammation with antigen-presenting cells in the Damaged weight loss
and damage (villous atrophy) to lamina propria via HLA-DQ2 and -DQ8 • Unexplained iron-deficiency
the lining of the small intestine. and activates gluten-sensitive T cells Fig. 1 Lining of small bowel vs lining of small bowel with anaemia, or other unspecified
Coeliac disease is associated with • The resultant inflammatory reaction coeliac disease anaemia
dermatitis herpetiformis and other and release of mediators causes villous • Dermatitis herpetiformis
autoimmune disorders. atrophy and crypt hyperplasia (see Fig. 2)
(see Fig. 1)

Fig. 2 Dermatitis herpetiformis: itchy symmetrical eruption of vesicles and crusts over the
Definition Pathophysiology Clinical features extensor surfaces of the body associated with coeliac disease; it is caused by deposition
of IgA in the dermis

Coeliac disease Investigations

• If patient is already taking a gluten-free diet they should be asked,

if possible, to reintroduce gluten for at least 6 weeks prior to testing:
• Routine bloods FBC (anaemia), iron studies (iron deficiency),
vitamin B12 (↓), folate (↓), LFTs, calcium, albumin
Notes • Immunology TTG antibodies (IgA) are 1st line for diagnosis according
Coeliac disease
to NICE, endomyseal antibody (IgA), anti-gliadin antibody (IgA or IgG)
(not recommended by NICE), anti-casein antibodies are also found
in some patients
Complications Management • Duodenal/jejunal biopsy (see Fig. 3) Villous atrophy, crypt hyperplasia,
↑intraepithelial lymphocytes, lamina propria infiltration with lymphocytes
• Dual energy X-ray absorptiometry (DEXA) scan Performed at diagnosis
• The following patients should be screened for coeliac due to increased risk of osteoporosis
• Anaemia (iron, folate and vitamin
B12 deficiency) disease: autoimmune thyroid disease, dermatitis
• Hyposplenism herpetiformis, irritable bowel syndrome, type 1
• Osteoporosis, osteomalacia diabetes mellitus, 1st-degree relatives of patients with
• Lactose intolerance coeliac disease
• Malignancy: enteropathy-associated • Lifelong strict gluten-free diet (GFD) is the only known
T-cell lymphoma of small intestine, effective treatment
oesophageal cancer
• Subfertility, unfavourable pregnancy
outcomes

Fig. 3 Duodenal biopsy showing complete villous atrophy, marked

crypt hyperplasia, intraepithelial lymphocytosis and inflammatory
infiltrate in the lamina propria

80 / Chapter 3 Gastroenterology: Coeliac disease Mind Maps for Medicine \ 81

 • The cause of Crohn’s disease is unknown but it is generally accepted to be caused • Smoking Risk is increased in smokers Intestinal • Eyes: conjunctivitis,
by an abnormal immune response to luminal antigens in genetically susceptible • Family history Family history is present in about 25–40% of children episcleritis, iritis
individuals • Infectious gastroenteritis Risk is increased 4-fold following • Diarrhoea (most common • Joints: large joint arthritis,
Crohn’s disease is a form of inflammatory • Microscopically, the initial lesion starts as a focal inflammatory infiltrate around an episode of infectious gastroenteritis symptom overall): can be bloody sacroiliitis, ankylosing
bowel disease (IBD). It is characterised by a the crypts, followed by ulceration of superficial mucosa; later, inflammatory cells • CARD15 gene mutation CARD15 gene encodes NOD2, but less common spondylitis
transmural granulomatous inflammation invade the deep mucosal layers and, in that process, begin to organise into non- a protein which is produced by intestinal epithelial cells and triggers • Abdominal pain: most prominent • Liver: fatty liver, primary
which commonly affects the terminal ileum caseating granulomas a protective inflammatory response which maintains intestinal symptom in children sclerosing cholangitis (rare),
and colon but may be seen anywhere from • Macroscopically, the initial abnormality consists of hyperaemia and oedema homeostasis; mutations of this gene significantly increase the risk • Weight loss and lethargy cholangiocarcinoma (rare)
the mouth to the anus. Unlike ulcerative of the involved mucosa; later, discrete superficial ulcers form over lymphoid • Appendectomy Risk increases early after an appendectomy • Perianal disease: e.g. skin tags or • Renal stones (oxalate)
colitis (UC), there may be unaffected bowel aggregates and are seen as red spots or mucosal depressions and decreases to that of the general population about 5 years ulcers • Osteomalacia
between areas of active disease (skip lesions). • Transmural inflammation results in thickening of the bowel wall and narrowing postoperatively • Malnutrition
There may also be a number of extra-intestinal of the lumen; as the disease progresses, it is complicated by obstruction or deep • Drugs NSAIDs and COCP may increase the risk of relapse or Extra-intestinal • Amyloidosis
features present. ulceration leading to fistulisation by way of the sinus tracts penetrating the exacerbation of IBD, but the absolute risk is low
serosa, microperforation, abscess formation, adhesions and malabsorption • Clubbing
• Skin: erythema nodosum,
pyoderma gangrenosum
Definition Pathophysiology Risk factors

Clinical features

Crohn’s disease

Notes
Crohn’s disease

Complications Management
Investigations
Conservative Surgical
• Psychosocial impact
• Abscesses (intestinal wall or • Smoking cessation: patient should be encouraged • Around 80% of patients with Crohn’s
adjacent structures) to stop smoking as smokers more likely to have disease will eventually have surgery • Bloods FBC, CRP/ESR (↑ in active disease), U&Es, LFTs,
• Intestinal strictures significant symptoms, higher chance of relapse and • Often indicated if there is failure vitamin B12, folate, ferritin, vitamin D
• Fistulas more likely to have complications post surgery of medical therapy or to manage • Stool Culture and microscopy, faecal calprotectin (a small
• Anaemia: iron deficiency • Specialist nurse advice and support complications e.g. intestinal obstruction, calcium-binding protein) – the concentration in faeces has
(blood loss or nutritional • Dietary advice: there are no specific foods strictures, fistulas and perianal disease been shown to correlate well with the severity of intestinal
deficiency), vitamin B12 or that patients should avoid; patients should be • In patients who have isolated terminal inflammation and can therefore be used for diagnosis and
folate deficiency (↓absorption), encouraged to have a balanced diet; they may ileum disease, however, an ileocaecal monitoring
or anaemia of chronic disease require supplements such as iron, B12, folate or resection can be preferred to medication • AXR Used in patients with acute severe colitis; helps to rule
• Malnutrition, failure to thrive calcium in the early stages to prolong and out toxic megacolon or bowel obstruction
and delayed puberty (in • Psychological support: assess the impact of maintain remission • Ileocolonoscopy Identifies macroscopic features and also
children) symptoms on daily functioning, and assess for biopsies from the terminal ileum to look for microscopic
• Colorectal and small bowel associated anxiety and/or depression Other treatments evidence of Crohn’s disease are 1st line to establish
cancer diagnosis (contraindicated in an acute flare)
Medical (see Table 2) • Enteral feeding with an elemental • Barium enema High sensitivity and specificity for
diet or polymeric can be used to induce examination of the terminal ileum, strictures (‘Kantor’s string
• Glucocorticoids are generally used to induce remission in addition to or instead of sign’), proximal bowel dilatation, ‘rose thorn’ ulcers and
remission other measures, particularly if there is fistulae
• Aminosalicylates (5-ASAS), e.g. mesalazine, are concern regarding the side effects of • CT colon To assess both mural and extramural
used 2nd line to glucocorticoids to induce remission steroids e.g. in young children; it is not manifestations of IBD
but are not as effective effective in maintaining remission • MRI colon To assess both mural and extramural
• Immunosuppressant, e.g. azathioprine or • Total parenteral nutrition is manifestations of IBD
mercaptopurine, may be used as an add-on appropriate adjunctive therapy in • Pelvic MRI For perianal disease
medication to induce remission and are often used complex, fistulating disease • Oesophagogastroduodenoscopy (OGD) Recommended
1st line to maintain remission for patients with upper GI symptoms

82 / Chapter 3 Gastroenterology: Crohn’s disease Mind Maps for Medicine \ 83

 Crohn’s disease notes Notes
Crohn’s disease

Table 1 Ulcerative colitis vs Crohn’s disease

Ulcerative colitis Crohn’s disease

Site Inflammation starts at the rectum and never spreads Can occur anywhere in the GI tract from the mouth to
beyond ileocaecal valve the anus but most commonly affects the terminal ileum

Macroscopic pathology • No inflammation beyond the submucosa • Can extend through all layers of the bowel wall
• Continuous pattern of inflammation • Intermittent pattern of inflammation (skip lesions)

Microscopic pathology • No inflammation beyond submucosa • Transmural inflammation

• Goblet cell depletion and crypt abscesses • Granulomas present
• Increased goblet cells

Clinical features • Usually causes bloody diarrhoea • Does not usually cause bloody diarrhoea
• Abdominal pain typically in left lower quadrant, • Weight loss and upper GI symptoms more prominent
tenesmus and urgency • Mouth ulcers, perianal disease

Risk factors Smoking reduces risk of disease Smoking increases risk of disease

Endoscopy findings Ulcers and pseudopolyps Deep ulcers, skip lesions – ‘cobble-stone' appearance

Complications Complications include haemorrhage and toxic Complications include bowel obstruction, abscess
megacolon and fistulas

Extra-intestinal features Primary sclerosing cholangitis is more common in UC Gallstones are more common in Crohn’s disease

Table 2 Pharmacological agents used in Crohn’s disease

Glucocorticosteroids • e.g. Prednisolone (oral), hydrocortisone (IV), budesonide (oral)

• Usually reserved for active Crohn’s disease to help induce remission
• They should not routinely be used for maintenance

Aminosalicylates (5-ASAS) • e.g. Mesalazine, sulfasalazine

• These are used 2nd line to glucocorticoids to induce remission but are not as effective
• They can be considered for maintaining remission in patients with previous surgery

Immunosuppressants • e.g. Azathioprine, mercaptopurine, methotrexate, ciclosporin

• Can be used with glucocorticosteroids to induce remission and can be useful as steroid-sparing agents to
maintain remission
• Azathioprine or mercaptopurine used 1st line to maintain remission with methotrexate 2nd line
• Can be used long term but require frequent monitoring particularly for liver and bone marrow toxicity

Biological agents • e.g. Infliximab, adalimumab, vedolizumab

• Infliximab is an anti-TNF-alpha drug used in refractory disease and fistulating Crohn's disease
• Adalimumab is another anti-TNF-alpha drug that can be used if there is intolerance to infliximab
• Vedolizumab is an anti-integrin biological agent that is more gastrointestinally targeted; it is recommended by
NICE as an option for treating moderate to severe active Crohn's disease only if a TNF-alpha inhibitor has failed,
cannot be tolerated or is contraindicated

Metronidazole Often used for isolated peri-anal disease

84 / Chapter 3 Gastroenterology: Crohn’s disease notes Mind Maps for Medicine \ 85

 • 95% of gastric carcinomas are adenocarcinomas; 64% of carcinomas are situated in the Risk factors Symptoms
prepyloric region
• There are 3 morphological forms: • Gastric cancer is the fourth most common cancer worldwide • Epigastric pain
• Fungating tumours Usually polypoid and may grow to be very large; tend to have a and the leading cause of cancer death in Japan • Dyspepsia
better prognosis • It is only the 15th most common cancer in the UK but the 4th • Nausea/vomiting
• Malignant ulcers Broad-based tumours with a necrotic centre commonest cause of death from cancer in the UK • Haematemesis
• Infiltrating carcinoma Spread widely beneath the mucosa and invade the muscular • Weight loss
layer; large mucin droplets displace the nuclei laterally producing the so-called ‘signet Epidemiology • Anorexia
Gastric cancer refers to a neoplasm ring appearance’; very poor prognosis • Dysphagia
• Metastatic spread can occur directly to adjacent structures, lymphatically via coeliac nodes, • Increasing age (95% of gastric cancers occur in those • Symptoms of anaemia
that can develop in any portion of
transcoelomically and haematogenously (via the portal or systemic vessels) aged >55 years)
the stomach and may spread to the
• More common in men than women (UK ratio is 1.8:1) Signs
lymph nodes and other organs.
• It is strongly associated with poor socio-economic status
• Helicobacter pylori can double the risk of gastric cancer in • Epigastric mass
infected individuals • Troisier’s sign: the clinical finding of a
Definition Pathophysiology • Diet: low levels of fresh fruit and vegetable consumption, high hard and enlarged left supraclavicular Fig. 1 Troisier’s sign
salt and consumption of preserved foods increases the risk node (Virchow node), considered
of gastric cancer a sign of metastatic abdominal
• Smoking malignancy (see Fig. 1)
• Atrophic gastritis, pernicious anaemia, post partial-gastrectomy • Hepatomegaly, jaundice ascites
• Ménétrier’s disease (liver spread)
• Familial risk: a 2- to 3-fold increased risk to 1st-degree relatives • Acanthosis nigricans: brown, poorly
of gastric cancer patients; link between E-cadherin gene defined, velvety hyperpigmentation
mutations and some familial gastric cancers of the skin (see Fig. 2)
• Blood group A • Dermatomyositis
• Hypogammaglobulinaemia • Signs of anaemia
Gastric cancer
Risk factors and epidemiology Fig. 2 Acanthosis nigricans

Clinical features

Management Prognosis and staging Investigations

Notes
Gastric cancer
Surgery • TNM staging is used to stage gastric Bloods FBC (iron-deficiency
cancer anaemia is common), LFTs (may be
• Surgery is the treatment of choice for gastric • Overall survival rate is 15% in the UK deranged in liver metastases)
cancer and the only potentially curative • 11% of people live for at least 10 years Upper GI endoscopy Investigation
treatment modality • Younger people tend to survive longer of choice to identify tumour(s);
• Distal (antral) tumours should be treated by biopsies can be taken and small
subtotal gastrectomy and proximal tumours by lesions evaluated more fully than
total gastrectomy radiological studies
Barium meal Rarely used in UK as
Chemotherapy and radiotherapy superseded by endoscopy but it is
less invasive
• Perioperative combination chemotherapy is the
CT abdomen Mainly used for
treatment of choice for localised gastric cancer
staging
throughout the UK and most of Europe
Stool testing Faecal occult blood
• Adjuvant chemotherapy without radiotherapy
test positive in the vast majority of
after surgery is not currently standard
subjects
procedure in the UK but may be helpful in high-
risk patients
• 5-fluorouracil (5-FU) is the most effective
chemotherapeutic agent; a combination of
5-FU with other agents is superior to single-
agent treatment

86 / Chapter 3 Gastroenterology: Gastric cancer Mind Maps for Medicine \ 87

 • GORD includes all the consequences of reflux of acid or other irritants from • Stress and anxiety • Heartburn Related to meals, lying down, stooping and
the stomach into the oesophagus • Smoking Oesophagus
Oeso gus straining; relieved by antacids
• The main cause of GORD is incompetence of the antireflux barriers at the • Alcohol excess Diaphragm
Diap ragm • Retrosternal discomfort
oesophagogastric junction; the antireflux barriers include two ‘sphincter’ • Fatty foods and coffee • Acid brash Regurgitation of acid or bile
mechanisms: the lower oesophageal sphincter (LOS), and the crural • Drugs that decrease LOS • Water brash (excessive salivation)
diaphragm that functions as an external sphincter pressure e.g. tricyclic • Odynophagia (pain on swallowing)
‘Dyspepsia‘ is used to describe a complex of upper • Reflux occurs when LOS pressure is lower than the intragastric pressure antidepressants, • Chest pain/epigastric pain
b)
gastrointestinal (GI) tract symptoms which are typically • Mucosal defence mechanisms may be overcome by prolonged exposure of anticholinergics, nitrates and a) • Bloating
present for ≥4 weeks, including upper abdominal the oesophageal mucosa to acid which may lead to severe and complicated calcium-channel blockers Fig. 1 There are two types of hiatus hernia:
• Chronic hoarseness, chronic cough and asthmatic
pain or discomfort, heartburn, acid reflux, nausea oesophagitis • Pregnancy (a) Sliding hiatus hernia The gastro-oesophageal junction
symptoms (atypical symptoms)
and/or vomiting. • Hiatus hernia (see Fig. 1) slides up into the thoracic cavity (85–95% of cases)
• Family history (b) Rolling hiatus hernia (paraoesophageal) The gastro-
Gastro-oesophageal reflux disease (GORD) is
usually a chronic condition where there is reflux of Pathophysiology oesophageal junction remains in place but a part of the Clinical features
gastric contents (particularly acid, bile and pepsin) stomach herniates into the chest next to the oesophagus
back into the oesophagus, causing predominant (5–15% of cases); many are mixed, with a sliding
symptoms of heartburn and acid regurgitation. component also

Definition Risk factors

Gastro-oesophageal Notes
reflux disease Gastro-oesophageal reflux disease

Management
Investigations
Conservative
• FBC To exclude anaemia
• Weight loss • Endoscopy Investigation of choice
• Smoking cessation • CXR Hiatus hernias may be seen as a
• Reduce alcohol intake soft tissue opacity with or without an
• Elevated head of the bed at night air-fluid level
• Take small, regular meals • Barium swallow Useful for diagnosing
• Avoid hot drinks, alcohol and eating 3 or 4h before bed hiatus hernia
• Avoid offending drugs • Oesophageal pH monitoring To
assess if symptoms coincide with acid
Pharmacological in the oesophagus
• Antacids e.g. Aluminium hydroxide, sodium bicarbonate, calcium carbonate
• H2 receptor antagonists e.g. Ranitidine or cimetidine
• PPIs e.g. Omeprazole, lansoprazole and pantoprazole Complications
Surgical
• Oesophageal ulcers
• Fundoplication surgery Strengthening the LOS by wrapping the gastric • Oesophageal haemorrhage
fundus around it • Anaemia (usually 2° to chronic blood
• Laparoscopic insertion of a magnetic bead band Involves putting a loss from severe oesophagitis)
small flexible band of interlinked magnetic beads around the outside of the • Oesophageal stricture
lower oesophagus, just above the stomach • Aspiration pneumonia
• Barrett’s oesophagus
• Oesophageal adenocarcinoma
• Oral problems: dental erosions,
gingivitis and halitosis

88 / Chapter 3 Gastroenterology: Gastro-oesophageal reflux disease Mind Maps for Medicine \ 89

 • In most cases HH is caused by a mutation of the • Pituitary Fatigue, loss of libido, erectile dysfunction, testicular atrophy
Chronic fatigue
HFE gene on the short arm of chromosome 6 • Pancreas Diabetes mellitus Common in haemochromatosis
• The known mutations of HFE are C282Y and H63D; the • Skin ‘Bronze’ skin pigmentation (see Fig. 2) Common in non-HFE juvenile Hypopituitarism
haemochromatosis
C282Y mutation is most common in White populations • Joints Arthritis (especially of the hands)
• HFE protein is responsible for regulation of the primary • Cardiac Arrhythmias, cardiomyopathy, heart failure
iron regulatory hormone, hepcidin • Liver Stigmata of chronic liver disease, hepatomegaly, cirrhosis,
• Mutation of HFE results in decreased hepcidin hepatocellular deposition • Hepatomegaly • Cardiac rhythm
production in response to elevated iron levels resulting • Cirrhosis disorders
in unregulated control of iron levels • Hepatocellular • Cardiac failure
carcinoma
Hereditary haemochromatosis • Increased intestinal absorption of iron causes
(HH) is a multi-organ autosomal accumulation of iron in tissues, especially the liver,
recessive disorder of iron which may lead to organ damage; other organs that • White nails
• Flat nails
absorption and metabolism resulting may be affected by iron deposits include the pituitary • Koilonychia
in iron accumulation. gland, pancreas, joints, heart, skin and gonads Diabetes mellitus

• Joint pain
Definition Pathophysiology • Osteoporosis
• Melanoderma
• Skin dryness
Fig. 2 ‘Bronze’ skin pigmentation in HH
Fig. 1 Clinical manifestations of HH

Clinical features (see Fig. 1)

Notes
Hereditary Hereditary haemochromatosis

haemochromatosis

Management Investigations

• Phlebotomy Should be carried out by removing

400–500ml of blood (200–250mg iron) weekly or
every 2 weeks
• Monitoring C282Y homozygotes without
evidence for iron overload could have ferritin
monitored annually and treatment instituted Screening tests Further investigations
when the ferritin rises above normal; haemoglobin
• Iron studies Transferrin saturation • Molecular genetic testing This is for
and ferritin levels must be monitored regularly
(>55% in men or >50% in women), C282Y and H63D mutations, but now
• Diet Iron-containing vitamin preparations and
ferritin (raised) and iron (raised), rarely required because genetic testing
iron-supplemented foods such as breakfast cereals
TIBC (low) for HFE mutations is very reliable
should be avoided
• LFTs May be deranged but often • Liver biopsy With Perl’s stain, now
• Iron chelation therapy Can be used as a
normal even with cirrhosis rarely required because genetic testing
2nd-line option in patients who are intolerant of
• CRP Helps exclude inflammation as for HFE mutations is very reliable
phlebotomy e.g. deferoxamine or deferasirox
a cause for raised ferritin as ferritin is • MRI liver Useful non-invasive
• Genetic testing of family members Genotyping
also an acute-phase reactant method to detect and quantify
to detect the HFE gene mutations and iron studies
• Joint X-ray Characteristically shows hepatic iron excess
should be performed on 1st-degree relatives of
chondrocalcinosis • Fasting glucose/HbA1c To detect
affected individuals
diabetes
• Immunisation Patients with HFE-HC could
• ECG/ECHO Detect arrhythmias and
be immunised against hepatitis A and B while
heart failure
iron-overloaded

90 / Chapter 3 Gastroenterology: Hereditary haemochromatosis Mind Maps for Medicine \ 91

 • There is no structural lesion or organic of pain at lower thresholds than those • Genetic Twin studies and family studies confirm • Abdominal pain Often relieved by defecation or passage of wind
cause for IBS; however, it is thought without it, suggesting some role of the familial aggregation of IBS • Abdominal bloating and distension
to be associated with abnormal central pain processing system • Enteric infection e.g. Following gastroenteritis • Excess flatus
smooth muscle activity ± visceral • IBS has been linked with increased levels • Gastrointestinal inflammation e.g. Secondary • Change in bowel habit
hypersensitivity, and abnormal central of psychiatric distress and poor coping to inflammatory bowel disease • Altered stool passage Subtypes of IBS can be classified according to the
processing of painful stimuli strategies • Dietary factors e.g. Excessive alcohol, caffeine, spicy predominant stool pattern: IBS with constipation, IBS with diarrhoea, and
• Furthermore, there is evidence of • There may be a genetic component to and fatty foods (up to 90% of people report that food mixed IBS with alternating diarrhoea and constipation
abnormal bowel transit time in IBS as there can be aggregation of the triggers symptoms) • Sensation of incomplete evacuation of stool
Irritable bowel syndrome (IBS) is a affected individuals, suggesting condition in families • Drugs e.g. Antibiotics • Rectal passage of mucus
chronic, relapsing, and often lifelong possible disturbed GI motility • Psychosocial e.g. Associated stress, anxiety
disorder of the lower GI tract, with no • Balloon distension of the bowel in and/or depression
apparent structural or biochemical affected individuals leads to perception
cause. Clinical features
Risk factors
Definition Pathophysiology

Irritable bowel syndrome

Investigations
Diagnostic criteria (NICE, CG61)
All investigations in IBS are normal. The following
Conservative Management Pharmacological treatments investigations should be carried out in all patients: The diagnosis should be considered if the patient has had the following
• FBC, ESR, CRP, LFTs Useful tests to screen for for at least 6 months:
Diet: • Loperamide 1st-line treatment for diarrhoea inflammation and other pathology • Abdominal pain and/or
• Regular meals, avoiding long gaps between meals • Antispasmodics e.g. Mebeverine should be used • Coeliac disease screening e.g. Tissue • Bloating and/or
and avoid rushing meals as required for abdominal pain and spasms transglutaminase • Change in bowel habit
• Drink plenty of fluids (at least 8 cups/day) but restrict • Peppermint oil Shown to be effective as an • CA-125 For women with symptoms which could be A positive diagnosis of IBS should be made if the patient has abdominal
tea/coffee antispasmodic and for bloating, with very few ovarian cancer pain relieved by defecation or associated with altered bowel frequency
• Reduce intake of alcohol and fizzy drinks adverse effects • Faecal calprotectin For those with symptoms which or stool form, in addition to 2 of the following 4 symptoms:
• Limitation of high-fibre foods (e.g. wholemeal flour • Laxatives May be used as required for constipation; could be IBD • Altered stool passage (straining, urgency, incomplete evacuation)
or bran) and resistant starches and limit fresh fruits linaclotide can be used as an alternative when other The following tests are NOT required to confirm IBS in • Abdominal bloating, distension, tension or hardness
to 3 portions per day; those with constipation as a laxatives have not worked; lactulose should be avoided those who meet the diagnostic criteria but should be • Symptoms made worse by eating
predominant symptom may need to increase fibre • Antidepressants TCAs and SSRIs; NICE guidelines done if diagnosis unclear: • Passage of mucus
intake however support the use of an SSRI only if a low-dose TCA • TFTs (Features such as lethargy, nausea, backache and bladder symptoms
• Avoid sorbitol with diarrhoea has not been effective; treatment should be started • US abdomen may also support the diagnosis)
• Consider increasing oats and linseeds for wind at a low dose e.g. 10mg amitriptyline and increased • Colonoscopy/sigmoidoscopy/barium enema
• Consider referral to dietitian for those who find diet if necessary • Faecal occult blood
plays a significant role in their symptoms for advice • Antibiotics May have a role in IBS by altering the • Faecal ova and parasite tests
about exclusion diets bacterial composition of the GI tract e.g. short-course • Helicobacter pylori screening e.g. through a stool test
therapy with rifaximin or neomycin
Lifestyle and physical activity: Other therapies
• Approx. 75% of patients are helped by explanation
and symptomatic relief • Psychological intervention If symptoms do
• Encourage increased physical activity, exercise and not respond to pharmacological treatments after
time for relaxation 12 months and for those who develop refractory IBS:
consider referral for cognitive behavioural therapy
(CBT) or hypnotherapy

92 / Chapter 3 Gastroenterology: Irritable bowel syndrome Mind Maps for Medicine \ 93

Jaundice (also known as icterus) Pre-hepatic Hepatic Post-hepatic • Jaundice can be caused by a wide variety • Intra-hepatic In the liver, unconjugated
Red cell Haem protein Bone marrow
describes the yellow pigmentation of disorders which range from benign to bilirubin is then conjugated and can be destruction catabolism erythropoiesis
of the skin, sclera, and mucous • Haemolytic anaemias: sickle • Viral hepatitis: including • Gallstones life-threatening conditions excreted in bile
membranes as a result of raised cell, thalassaemia, hereditary hepatitis A–E, CMV and EBV • Surgical strictures • Bilirubin is a breakdown product of haem molecules • Post-hepatic Soluble bilirubin is
plasma bilirubin (see Fig. 1). spherocytosis, glucose-6- • Alcohol • Biliary atresia in red blood cells and other proteins such as transported through the liver and Bilirubin
Normal serum bilirubin is approx. phosphate dehydrogenase • Cirrhosis • Cholangiocarcinoma myoglobin cystic ducts in bile and then stored
3–20μmol/L; clinical jaundice may deficiency, haemolytic • Non-alcoholic fatty liver disease • Extra-hepatic malignancy: • The causes of jaundice can be broadly classified in the gallbladder or passes into Circulating albumin-
not become apparent until serum by the stages of bilirubin metabolism (see Fig. 2); the duodenum; in the intestine, bilirubin (unconjugated)
uraemic syndrome (NAFLD) e.g. pancreatic cancer
bilirubin is >35μmol/L. • Drugs: e.g. methyldopa and • Autoimmune liver disorders: e.g. • Pancreatitis dysfunction at any of these 3 phases can lead to some bilirubin is excreted in the
sulfasalazine primary biliary cholangitis (PBC), • Parasitic infection: including jaundice: stool (as stercobilinogen) and the
(Circulating
• Severe rhabdomyolysis autoimmune hepatitis hydatid disease, liver fluke • Pre-hepatic Haem molecules are degraded in rest is metabolised by gut flora into Hepatic conjugated
macrophages via biliverdin to bilirubin; this occurs urobilinogen which is reabsorbed conjugation
• Malaria • Metabolic causes of intra-hepatic and roundworms bilirubin)
• Gilbert syndrome: an jaundice • Primary sclerosing cholangitis mainly in the spleen and liver; bilirubin is then and excreted by the kidneys Conjugated
autosomal recessive • Drugs: e.g. paracetamol overdose, • Cholestasis of pregnancy bound to plasma albumin and transported to the bilirubin
condition of defective pyrazinamide, rifampicin and • Drugs: e.g. co-amoxiclav, liver for conjugation
bilirubin conjugation due to a isoniazid flucloxacillin, oral contraceptives,
deficiency of UDP glucuronyl • Leptospirosis hormone replacement therapy
transferase; no treatment is • Liver malignancy and corticosteroids
Fig. 1 Jaundiced patient required • Wilson’s disease Pathophysiology
• Crigler–Najjar syndrome
Causes
Definition Bacterial deconjugation

Faecal stercobilinogen

Fig. 2 Metabolism of bilirubin

Jaundice
Management Notes
Jaundice

Treat underlying cause

Diagnosis (see Table 1)

Bloods • Hepatitis serology, ANA (+ve in 20–50% of patients with PBC),

ASMA (+ve in autoimmune hepatitis), AMA (+ve in 90–95% of
• ALT, AST, bilirubin (including bilirubin split, see Table 1), gamma-GT, ALP, PBC), serum immunoglobulins (↑IgG in acute hepatitis, ↑IgM in
albumin, clotting, FBC including reticulocyte count and blood smear autoimmune disease, PBC or chronic infection)
(to detect haemolysis), LDH (↑ in haemolysis) • Viral serology for hepatitis A, B, C, E; CMV
Table 1 Distinguishing between pre-hepatic, hepatic and post-hepatic jaundice • Monospot test for EBV
• Alpha fetoprotein: ↑ in hepatocellular carcinoma
Clinical Pre-hepatic Hepatic Post-hepatic • Caeruloplasmin: ↓ in Wilson’s disease
features/LFTs • Alpha-1 antitrypsin (↓ in alpha-1 antitrypsin deficiency)

Urine Normal Dark Dark Urine

appearance
Urinary bilirubin and urobilinogen:
Stool Normal Pale Pale • ↑Urinary bilirubin ↓urobilinogen is suggestive of obstructive
appearance jaundice
• → or ↑urinary bilirubin with ↑urobilinogen suggests
Total bilirubin ↑ ↑ ↑ hepatocellular failure or increased red cell breakdown

Conjugated ↑ ↑ ↑ Imaging
bilirubin
• Abdominal US: to detect liver abnormalities,
hepatosplenomegaly and gallstones
Unconjugated ↑ ↑ →
• CT abdomen (as with US but more detailed)
bilirubin
• Magnetic resonance cholangiopancreatography (MRCP): test of
choice in obstructive jaundice
ALP Normal ↑ ↑
• Liver biopsy, laparotomy: may ultimately be required to make the
diagnosis in some cases of jaundice

94 / Chapter 3 Gastroenterology: Jaundice Mind Maps for Medicine \ 95

 • Over 8000 new oesophageal cancers are • Smoking • SCC Mainly found in the upper two-thirds of the oesophagus and thought • Dissemination of the tumour may occur in 3 ways:
diagnosed each year in the UK • Alcohol to develop from squamous dysplasia/intraepithelial neoplasia • Direct spread Occurs both laterally, through the component layers of
• The incidence of oesophageal carcinoma • GORD • Dysplastic squamous epithelium Characterised by cytological the oesophageal wall, and longitudinally within the oesophageal wall
varies considerably with geographical • Barrett’s oesophagus abnormalities which are usually confined to the epithelium; invasion of • Via lymphatics Common
location; high rates in China and Iran • Achalasia these neoplastic squamous cells into lamina propria and deeper layers • Haematogenous spread May involve a variety of different organs
(SCC) have been directly linked to the • Plummer–Vinson syndrome results in invasive oesophageal SCC e.g. liver, lungs, brain and bones
preservation of food using nitrosamines • Tylosis and Paterson–Brown–Kelly • Adenocarcinoma usually arises from Barrett’s oesophagus in the lower
• Adenocarcinoma is seen more frequently syndrome (SCC) third of the oesophagus where normal distal squamous epithelial lining
in Caucasian populations • Diets rich in nitrosamines (SCC) has been replaced by metaplastic columnar epithelium (see Fig. 1).
• Coeliac disease, scleroderma (rare)
Oesophageal cancer is cancer
of the oesophagus with the two
main types being squamous
cell carcinoma (SCC) and Epidemiology Risk factors GORD
Metaplasia/Barrett’s
Low-grade dysplasia High-grade dysplasia Adenocarcinoma
oesophagus
adenocarcinoma.

Definition
Fig. 1 Stages of oesophageal adenocarcinoma

Pathophysiology

Oesophageal cancer and

other causes of dysphagia

Notes
Oesophageal cancer and other causes of dysphagia

Management Investigations Clinical features (VBAD)

• Involvement of a multidisciplinary team including a gastroenterologist, • Bloods FBC, U&Es, LFT, glucose, CRP • Vomiting
specialist nurse, dietitian, speech and language therapy • Upper GI endoscopy and biopsies The gold standard for • Blood loss Melaena, haematemesis
• Operable disease is best managed by surgical resection diagnosis (see Fig. 2) • Anorexia Plus weight loss
• The two most commonly performed surgeries are the transhiatal • Barium swallow Useful in diagnosing benign motility disorders • Dysphagia Most common presenting symptom
oesophagectomy and the transthoracic oesophagectomy, also known but has no place in the assessment of tumours • Other symptoms: odynophagia, hoarseness,
as the Ivor Lewis procedure • CT Chest, abdomen and pelvis for staging cough, retrosternal pain, intractable hiccups,
• In addition to surgical resection many patients will be treated with adjuvant • Endoscopic US For local staging if CT does not show metastatic lymphadenopathy
chemotherapy disease
• For non-operable tumours, treatment is mostly palliative • Staging laparoscopy To detect occult peritoneal disease
• Palliative options include stent insertion, radiotherapy, chemotherapy, • PET CT Performed in those with negative laparoscopy
laser therapy or a combination of these
• Nutritional support in the form of nasogastric tube, percutaneous To the
endoscopic gastrostomy (PEG) or radiologically inserted gastrostomy stomach
(RIG) insertion
Staging and prognosis

• TNM staging is used for oesophageal cancer

• The prognosis for oesophageal carcinoma varies
depending on the stage at presentation
Adenocarcinoma
tumour • The overall 5-year survival rate is 20–25% for all
stages
• The survival rates for adenocarcinoma and
squamous cell carcinoma are roughly the same

Fig. 2 Oesophageal adenocarcinoma on endoscopy

96 / Chapter 3 Gastroenterology: Oesophageal cancer and other causes of dysphagia Mind Maps for Medicine \ 97
 Oesophageal cancer and other causes of dysphagia notes

Specific causes of dysphagia

Causes of dysphagia by classification Achalasia Oesophagitis

Lung cancer • Primarily a disorder of motility of the lower oesophageal or cardiac sphincter • This may be caused by reflux (reflux oesophagitis) or infection such as
• Dysphagia of both liquids and solids from the start; usually causes heartburn candida (oesophageal candidiasis)
and regurgitation of food • Odynophagia often present but no weight loss and systemically well
Mediastinal • Diagnosis: • In oesophageal candidiasis there may be a history of HIV/AIDS or other risk
lymphadenopathy • Oesophageal manometry Excessive lower oesophageal sphincter tone factors such as chemotherapy or inhaled steroid use
which doesn’t relax on swallowing
• Barium swallow Shows grossly expanded oesophagus, fluid level, ‘bird’s
Extrinsic Retrosternal goitre
beak’ appearance
• CXR Wide mediastinum, fluid level
• Management options include intrasphincteric injection of botulinum toxin,
Aortic aneurysm
Heller cardiomyotomy and pneumatic (balloon) dilatation

Left atrial
enlargement Notes
Oesophageal cancer and other causes of dysphagia
Mechanical

Oesophageal cancer

Malignant Gastric cancer

Pharyngeal cancer

Benign oesophageal
Intrinsic
stricture

Oesophageal web
Benign
Causes of dysphagia

Schatzki rings
Achalasia

Pharyngeal pouch
Oesophageal wall Oesophageal spasm

Systemic sclerosis

CVA
Motility

Parkinson’s disease

Neurological Multiple sclerosis

Oesophagitis Brainstem pathology

Others

Globus hystericus Myasthenia gravis

Fig. 3 Causes of dysphagia

98 / Chapter 3 Gastroenterology: Oesophageal cancer and other causes of dysphagia notes Mind Maps for Medicine \ 99
 • In both types of peptic ulceration, gastric and • H. pylori infection: 95% of duodenal and • May be asymptomatic • Bloods FBC, U&Es, LFTs, iron studies
duodenal, there is an imbalance between 70–80% of gastric ulcers are associated • Epigastric pain: gastric ulcers typically give rise to pain 15–20min after • H. pylori testing Using a carbon-13 urea breath test or a stool
secretion and neutralisation of secreted acid with H. pylori infection meals, whereas duodenal ulceration typically causes pain 1–3h after a meal antigen test, or laboratory-based serology (not recommended)
• Most ulcers occur when the normal mechanisms • Drugs: NSAIDs (most common), aspirin, and may be relieved by food • Faecal occult blood To exclude blood loss
are disrupted by superimposed processes e.g. bisphosphonates, corticosteroids, • Epigastric tenderness • Upper GI endoscopy Diagnostic of peptic ulcers; endoscopy is
Helicobacter pylori infection and the ingestion of potassium supplements, SSRIs and • Nausea ± vomiting required if the patient is presenting for the first time and is above the
NSAIDs (see Risk factors) recreational drugs e.g. crack cocaine • Oral flatulence, bloating, distension and intolerance of fatty food age of 55 years or if there are any red flag features:
• Smoking • Heartburn (although more typically associated with GORD) • Anaemia (iron deficiency)
A peptic ulcer describes a breach • Excessive alcohol consumption • Pain radiating to back (posterior ulcer) • Weight loss
• Stress • Symptoms are relieved by antacids (not specific) • Progressive dysphagia
in the epithelium of the gastric or Pathophysiology • Zollinger–Ellison syndrome (rare): this • Haematemesis and melaena (if ulcer bleeding) • Persistent vomiting
duodenal mucosa that penetrates
the muscularis mucosae; it is hypersecretory state may be associated • Epigastric mass
confirmed on endoscopy. with multiple peptic ulcers, diarrhoea, • Chronic blood loss
weight loss and hypercalcaemia
Clinical features
Definition Investigations
Risk factors

Peptic ulcer disease

Notes
Peptic ulcer disease

Management Complications

• Haemorrhage: acute massive

Conservative Surgical
haemorrhage may be life-threatening
• Smoking cessation • Perforation: this may cause peritonitis
Indications:
• Reducing alcohol intake which may be life-threatening
• Failure of medical treatment • Gastric outlet obstruction: this may
• Stop offending drugs e.g. NSAIDs • Complications, e.g. recurrent result from strictures and stenosis of
haemorrhage, perforation,
Medical the pylorus and/or duodenum due to
outflow obstruction chronic inflammation and scarring
• Antacids e.g. Aluminium hydroxide, • A possibility of malignancy • Gastric malignancy: there is an
sodium bicarbonate increased risk in H. pylori positive
• H2 antagonists e.g. Ranitidine, Possible interventions: gastric ulcer disease
cimetidine • Vagotomy Removal of all or
• PPIs e.g. Lansoprazole, omeprazole, some of the branches of the
pantoprazole vagus nerve in order to prevent
• H. pylori triple eradication therapy – excessive acid secretion; it is the
offer people who test positive for surgical treatment of choice for
H. pylori a 7-day, twice-daily course of chronic duodenal ulceration
treatment with: • Partial gastrectomy Remove
• A PPI, e.g. omeprazole an ulcer, or remove a gastrin-
20mg BD, and secreting mucosa; a Billroth I
• Amoxicillin 1g, and type of partial gastrectomy is the
• Either clarithromycin 500mg or standard type of operation for a
metronidazole 400mg chronic gastric ulcer

100 / Chapter 3 Gastroenterology: Peptic ulcer disease Mind Maps for Medicine \ 101
 • The typical patient is a • Jaundice (cholestatic) with
woman aged 30–65 years pale stool, dark urine
• Presentation of PBC may vary
greatly from asymptomatic Signs
and slowly progressive to • Sjögren syndrome (seen in up to 80% of patients)
Primary biliary cholangitis (PBC; symptomatic and rapidly • Hepatomegaly (25%) • Rheumatoid arthritis
previously known as primary biliary evolving • Hyperpigmentation (25%) • Seronegative arthritis
cirrhosis) is an autoimmune disease • Splenomegaly (15%) • Systemic sclerosis Diagnosis is based on 2 of the following 3 criteria
characterised by chronic progressive Symptoms • Jaundice (10%) • Thyroid disease being met:
destruction of intrahepatic bile ducts, • Xanthelasma (later • Coeliac disease 1. Biochemical evidence of cholestasis with evidence of
resulting in chronic cholestasis, • Fatigue stages due to • Hyperlipidaemia increased ALP activity
portal inflammation and fibrosis • Pruritus hypercholesterolaemia) • Gallstones 2. Presence of AMAs
which will eventually lead to cirrhosis • Right upper quadrant • Osteoporosis 3. Histological evidence of nonsuppurative destructive
and liver failure. pain/discomfort • Hepatocellular malignancy cholangitis and destruction of interlobular bile ducts

Definition Clinical features Associations Diagnostic criteria

Primary biliary cholangitis

Notes
Primary biliary cholangitis

Investigations

Bloods
Complications Staging Management • FBC (usually normal), ESR (may be raised), LFTs (ALP
characteristically raised, ALT/AST variable, bilirubin usually
• Renal tubular acidosis 1. Portal stage: portal inflammation • Cholestyramine: for itching normal but rises with disease progression)
• Hypothyroidism (in approx. 20%) and bile duct abnormalities • Fat-soluble vitamin • Lipids: cholesterol often raised
• Hepatocellular carcinoma 2. Periportal stage: periportal supplementation: A, D, E, K • Autoantibodies: anti-mitochondrial antibodies (AMA)
• Steatorrhoea and fat-soluble fibrosis, with or without periportal • Ursodeoxycholic acid: to slow M2 subtype are present in 98% of patients and are highly
vitamin deficiency inflammation disease progression and reduce specific; smooth muscle antibodies present in 30% of
• Complications of cirrhosis 3. Septal stage: septal fibrosis and need for liver transplantation patients; anti-nuclear antibodies (ANA) are present in
active inflammation • Liver transplantation: e.g. if about 35% of patients
4. Cirrhotic stage: nodules with bilirubin >100 (PBC is a major • Serum IgM (raised in >80%)
various degrees of inflammation indication); recurrence in graft • TFTs often reveal a lowered T4
can occur
Imaging

• US abdomen: to help detect extrahepatic obstructive

causes e.g. gallstones
• MRI or endoscopic retrograde cholangiography: to exclude
primary sclerosing cholangitis or other disorders that might
lead to chronic cholestasis
• Transient elastography: to evaluate the degree of
liver fibrosis
• Liver biopsy: confirms the diagnosis but not mandatory
to make the diagnosis (see Diagnostic criteria)

102 / Chapter 3 Gastroenterology: Primary biliary cholangitis Mind Maps for Medicine \ 103
 • The aetiology of PSC remains unknown, but it is thought to be • PSC is a rare condition with a prevalence of Symptoms
multifactorial, including genetic predisposition, exposure to an 1–16 per 100 000
environmental antigen and subsequent immunologic response • More common in males (2:1) • May be asymptomatic (presenting with abnormal LFTs or hepatomegaly)
• There is also an increased prevalence of HLA alleles A1, B8 and DR3 • Typical age of diagnosis is 4–5th decade of life • Jaundice and pruritus
in PSC • Approx. 80% of patients with PSC have • Right upper quadrant abdominal pain
• An autoimmune mechanism is also suggested as there is a inflammatory bowel disease (IBD) but only • Fatigue, weight loss, fevers and sweats
Primary sclerosing cholangitis (PSC) significant overlap between IBD and PSC approx. 5% of patients with IBD have PSC
is a rare chronic cholestatic disorder • There is also a marked increase in serum autoantibody levels Signs
of unknown cause characterised in patients with PSC including anti-neutrophil cytoplasmic
by inflammation and fibrosis of • Jaundice
intrahepatic and extrahepatic bile
antibodies (ANCA), anti-cardiolipin (ACL) antibodies and
anti-nuclear antibodies (ANA).
Epidemiology/risk factors • Hepatomegaly and splenomegaly
ducts, resulting in multifocal biliary • Signs of cirrhosis, portal hypertension or hepatic failure (advanced stage)
strictures.

Clinical features
Definition Pathophysiology

• Stage 1 Bile duct injury and portal inflammation with minimal fibrosis
• Stage 2 Expansion of portal tracts, periportal fibrosis, and inflammation
Staging (histological) • Stage 3 Fibrous septa, bridging fibrosis, more prominent ductopenia
• Stage 4 End-stage disease with secondary biliary cirrhosis

Primary sclerosing cholangitis

Notes
Primary sclerosing cholangitis

Investigations

Complications Management
Bloods

Biliary complications Medical • LFTs: often abnormal, ↑ALP and gamma-glutamyltransferase (GGT)
is most common; ALT and AST may be normal or elevated; bilirubin
• Biliary obstruction due to stones or strictures • Ursodeoxycholic acid may improve liver is raised in advanced PSC
• Acute or chronic cholangitis function and the patient’s symptoms but no • Serum albumin (may be low) and prothrombin time may rise as
definitive improvement in histology or mortality the disease progresses
Cirrhosis and associated complications • Cholestyramine helps to relieve pruritus; • IG, IgM and the serum globulin fraction levels may be elevated
rifampicin, naltrexone and sertraline may also • There may also be hypergammaglobulinaemia, raised IgM levels,
• Ascites be used for pruritus raised antibodies: perinuclear ANCA (p-ANCA), ACL antibodies
• Portal hypertension • Treat fat-soluble vitamin (A, D, E, K) and ANA
• Oesophageal varices deficiencies
• Liver failure Imaging and other tests
Surgical
Increased risk of cancers • US abdomen Useful initial investigation and may show bile duct
• Percutaneous transhepatic balloon dilatation and liver and splenic changes (not diagnostic however)
• Cholangiocarcinoma dilatation May be of benefit for dominant • MRCP The gold standard to visualise the intrahepatic and
• Colorectal cancer strictures extrahepatic bile ducts
• Liver transplantation Potentially curative • ERCP or transhepatic cholangiography May also have a role
and is indicated where there is hepatic failure, (though both are invasive)
ascites or oesophageal varices • MRI abdomen May help to exclude other disease and evaluate
the biliary system
• Liver biopsy Rarely diagnostic but may be useful for staging PSC
(see below)
• Colonoscopy and biopsies Should be performed in patients
diagnosed with PSC without known IBD and then repeated
annually in PSC patients with colitis
104 / Chapter 3 Gastroenterology: Primary sclerosing cholangitis Mind Maps for Medicine \ 105
 • Microscopically, acute and chronic • Family history: 1st-degree relatives of Intestinal
inflammatory cells infiltrate the people with UC have a 10–15% increased
lamina propria, crypt branching risk of developing the disease compared • Bloody diarrhoea
Ulcerative colitis (UC) is a and villous atrophy are present in with people without a family history • Urgency
chronic, relapsing-remitting, UC; neutrophils migrate through • Oral contraceptives: there is an association • Tenesmus
non-infectious inflammatory the walls of glands to form crypt between the use of oral contraceptives and • Abdominal pain, particularly in the left lower quadrant
disease of the GI tract. In abscesses; there is depletion of a) b) c) the development of IBD • Systemic upset: malaise, fever, weight loss
addition, UC has a number of goblet cells and mucin from gland • Not smoking: the risk of UC is reduced in
epithelium Fig. 1 (a) Ulcerative proctitis (inflammation is limited to smokers (in contrast to Crohn’s disease, Extra-intestinal
extra-intestinal manifestations.
• Unlike Crohn’s disease, there is no the rectum), (b) left-sided colitis (inflammation does not where smoking increases the risk)
It is sometimes difficult to
inflammation beyond submucosa extend proximally beyond the splenic flexure), (c) pancolitis Related to disease activity:
distinguish between UC and
• There is widespread ulceration with (inflammation extends proximally beyond the splenic flexure • Erythema nodosum
isolated colonic Crohn’s
disease. These patients preservation of adjacent mucosa to involve the entire colon) Risk factors • Aphthous ulcers
can be described as having which has the appearance of polyps • Episcleritis
indeterminate colitis. (‘pseudopolyps’) • Arthritis: pauciarticular, asymmetric
• The extent of colitis can be classified • Osteoporosis
into 3 types (see Fig. 1) Pathophysiology
Definition Unrelated to disease activity:
• Pyoderma gangrenosum
• Anterior uveitis
• Sacroiliitis
• Ankylosing spondylitis
• Clubbing
• Primary sclerosing cholangitis

Clinical features

Ulcerative colitis

Severity

Truelove and Witts’ severity index

Mild:
• <4 stools/day
Management • Small amounts of bloody stool
• No anaemia
Investigations • Pulse rate <90
• No fever
Inducing remission Maintaining remission
• Normal ESR/CRP
• Rectal (topical) aminosalicylates • Oral aminosalicylates e.g. mesalazine
• Bloods FBC, CRP (↑ in active disease), U&Es, LFTs Moderate:
or steroids: for distal colitis rectal • Azathioprine and mercaptopurine
• Stool Culture and microscopy, faecal calprotectin
mesalazine has been shown to be • Probiotics may prevent relapse in • 4–6 stools/day
(a small calcium-binding protein; the concentration
superior to rectal steroids and oral patients with mild-to-moderate • Increased blood in stools compared with above
of calprotectin in faeces has been shown to correlate
aminosalicylates disease • No anaemia
well with the severity of intestinal inflammation)
• Oral aminosalicylates
• Oral prednisolone is usually used Surgical • AXR Useful for acute severe colitis to assess extent of Complications • Pulse rate <90
colonic involvement; may show lack of faecal shadows, • No fever
2nd line for patients who fail to • Normal ESR/CRP
• Surgery is required in approx. 20% of mucosal thickening or toxic megacolon
respond to aminosalicylates
patients with UC • Erect CXR To rule out perforation in acute severe colitis • Psychological effects
• IV steroids for severe colitis Severe:
• Colectomy is a curative option for • Barium enema Loss of haustrations, superficial • Toxic megacolon
• Infliximab for moderate-to-severe
patients who fail to respond to, or ulceration, ‘pseudopolyps‘, ‘drainpipe colon’ (colon is • Colorectal cancer • ≥6 stools/day
UC, when disease is refractory
are intolerant of, medical treatment, narrow and short) • Venous thromboembolism • Visible blood in stools
to conventional treatment
or in those with complications such • Rigid or flexible sigmoidoscopy With biopsy • Osteoporosis (due to steroid use) • At least one feature of systemic upset: temperature
using corticosteroids and/or
as colorectal cancer • Colonoscopy with multiple biopsies 1st-line >37.8°C, pulse rate >90, anaemia, ESR >30
immunosuppressive agents
procedure for diagnosing colitis

106 / Chapter 3 Gastroenterology: Ulcerative colitis Mind Maps for Medicine \ 107
 Common History Examination

• Peptic ulceration • Abdominal pain • Pallor and other signs of anaemia

• Mucosal inflammation (oesophagitis, gastritis or duodenitis) • Bleeding: • Pulse: usually tachycardia
• Oesophageal varices • Haematemesis • Blood pressure: may be low
• Mallory–Weiss tear • Coffee-ground vomit • Postural hypotension
• Gastric carcinoma • Melaena • Cool extremities
• Coagulation disorders e.g. thrombocytopenia, warfarin • Haematochezia • Chest pain
• Loss of blood: shock, syncope, presyncope • Confusion
Rare • Features of underlying cause e.g. dyspepsia, • Delirium
Acute upper gastrointestinal weight loss, jaundice • Evidence of dehydration (dry mucosa, sunken eyes,
• Aortoenteric fistula (especially after aortic surgery) • Risk factors: alcohol, drugs (NSAIDs, skin turgor reduced)
bleeding (UGIB) is a gastroenterological
• Benign tumours: e.g. leiomyoma, carcinoid tumour, angioma corticosteroids) • Stigmata of liver disease may be present e.g. jaundice,
emergency with a mortality of 6–13%.
• Congenital: e.g. Ehlers–Danlos, Osler–Weber–Rendu, • Past history of bleeding gynaecomastia, ascites, spider naevi, hepatic flap
pseudoxanthoma elasticum • Signs of a tumour may be present e.g. nodular liver,
Definition abdominal mass, lymphadenopathy
• Subcutaneous emphysema and vomiting suggest
Causes Boerhaave syndrome (oesophageal perforation)
Assessment • Urine output should be monitored (oliguria is a sign
of shock)

Notes
Upper gastrointestinal bleed
Upper gastrointestinal bleed

Investigations

Bloods
Management
• FBC Haemoglobin to check for anaemia; often measured
serially to help assess trend
• Clotting Partial thromboplastin time (PTT), INR, activated
partial thromboplastin time (APTT)
Resuscitation Endoscopy • Cross-match
• LFTs To identify liver disease
• ABC approach, wide-bore IV cannula ×2 Endoscopy should be offered immediately after resuscitation in patients with a
• U&Es Urea is typically raised
• IV fluids e.g. normal saline or Hartmann’s solution while severe bleed. All patients should have endoscopy within 24h. The subsequent
waiting for blood products management then depends on whether the bleed is non-variceal or variceal. Endoscopy
• Patients with massive bleeding should be transfused
with blood, platelets and clotting factors in line with Non-variceal bleed: Endoscopy should be undertaken immediately after
local massive haemorrhage protocols resuscitation for unstable patients with severe acute UGIB.
• PPIs should not be routinely given but only to patients with stigmata of recent
• Red cells should be considered after loss of 30% of the
haemorrhage shown at endoscopy
circulating volume Imaging
• Bleeding during endoscopy should be stopped with a mechanical method (e.g.
• Fresh frozen plasma in those who have either a
clips) with or without adrenaline, thermal coagulation with adrenaline, fibrin or • Erect CXR: may identify perforated viscus
fibrinogen level <1g/L, or a PT (INR) or APTT >1.5×
thrombin with adrenaline • Erect and supine AXR: to exclude perforated viscus and ileus
normal
• Interventional radiology should be offered to unstable patients who re-bleed • Abdominal CT or US: To identify underlying disease and
• Platelets if actively bleeding and with platelet count of
after endoscopic treatment and urgent surgery if interventional radiology is not haemorrhage
less than 50 × 109/L
readily available • Angiography may be useful if endoscopy fails to identify
• Prothrombin complex concentrate in patients who are
taking warfarin and actively bleeding site of bleeding
• Terlipressin and prophylactic antibiotics should be Variceal bleed:
given to patients at presentation for variceal bleeds • Band ligation should be used for oesophageal varices and injections of N-butyl-
2-cyanoacrylate for patients with gastric varices
• Transjugular intrahepatic portosystemic shunts (TIPS) should be offered if
bleeding from varices is not controlled despite above measures

108 / Chapter 3 Gastroenterology: Upper gastrointestinal bleed Mind Maps for Medicine \ 109
 Upper gastrointestinal bleed notes Notes
Upper gastrointestinal bleed

Risk assessment
The following formal risk assessment scores are recommended
NICE for all patients with acute UGIB:
• The Blatchford score at first assessment and
• Rockall score after endoscopy

Blatchford score (initial assessment) The Rockall score (post-endoscopy)

Admission risk marker Score component value Variable Score 0 Score 1 Score 2 Score 3

Blood urea (mmol/L) Age (years) <60 60–79 ≥80

6.5–8.0 2
Shock No shock Pulse >100 SBP <100
SBP >100
8.0–10.0 3

10.0–25 4 Comorbidity Nil major CHF, IHD, Renal failure,

major liver failure,
>25 6 morbidity metastatic
cancer
Haemoglobin (g/dl) for men
Diagnosis Mallory– All other GI malignancy
12.0–12.9 1 Weiss diagnoses
10.0–11.9 3
Evidence of None Blood,
bleeding adherent clot,
<10.0 6
spurting vessel
Haemoglobin (g/dl) for women
A score <3 carries good prognosis but total score >8 carries high risk of mortality
10.0–11.9 1

<10.0 6

Systolic blood pressure (mmHg)

100–109 1

90–99 2

<90 3

Other markers

Pulse ≥100 (per min) 1

Presentation with melaena 1

Presentation with syncope 2

Hepatic disease 2

Cardiac failure 2

Scores range from 0 to 23, with higher scores corresponding to

increasing acuity and mortality

110 / Chapter 3 Gastroenterology: Upper gastrointestinal bleed notes Mind Maps for Medicine \ 111
 • Wilson’s disease is an autosomal recessive disorder • Serum caeruloplasmin (↓)
which involves mutation of the ATP7B gene on • Serum copper (often ↓) is counter- Notes
Wilson’s disease
chromosome 13 intuitive, but 95% of plasma copper is
• The fundamental defect is a failure of hepatic carried by caeruloplasmin
excretion of copper into bile: copper accumulates • 24-h urinary copper excretion (↑)
in the liver and secondarily suppresses the • LFTs may be deranged in liver disease
Wilson’s disease is a rare synthesis of caeruloplasmin (major copper- • ECG may indicate cardiac involvement
autosomal recessive disorder carrying protein in the blood) • Liver biopsy is often diagnostic but
characterised by excessive copper • Eventually, copper spills over into the circulation only required if clinical signs and
deposition in various parts of and deposits in the basal ganglia and brain, liver, non-invasive tests are inconclusive or
the body. kidneys, cornea and other organs if there is suspicion of additional liver
pathology
• MRI brain may show lesions in the
Definition Pathophysiology brain compatible with the neurological
features; it is common to find increased
density in the basal ganglia

Investigations

Wilson’s disease

Management Clinical features

Non-pharmacological some patients with neurological • Liver Hepatitis, cirrhosis, liver failure
Wilson’s disease experience severe • Neurological Basal ganglia degeneration, speech, behavioural
• Avoid foods containing high amount (often transient) deterioration and psychiatric problems, Parkinsonism, asterixis, chorea,
of copper e.g. chocolate, peanuts and of neurological symptoms dementia
mushrooms when starting treatment with • Cornea Kayser–Fleischer rings (dark rings that encircle
• Monitor urinary copper level to penicillamine the iris; Fig. 1)
indicate when the patient is back to • Trientine hydrochloride An • Kidneys Renal tubular acidosis (especially Fanconi syndrome)
within normal limits in response to alternative chelating agent which • Rheumatological Osteopenia and osteoarthritis
treatment; hepatic and renal function, is often used as 1st-line therapy • Cardiac Cardiac arrhythmias and cardiomyopathy
FBC and clotting should also be for patients with hepatic and • Other features include pancreatitis, hypoparathyroidism,
monitored neurological disease infertility, blue nails
• Annual slit-lamp examination • Zinc Prevents the absorption of
of Kayser–Fleischer rings should Note: The onset of symptoms is usually between 10 and 25 years.
copper but chelation treatment
document fading or disappearance if Children usually present with liver disease whereas the first sign of
should continue for 2–3 weeks
copper is being adequately removed disease in young adults is often neurological disease.
after it has been started, as the
• Genetic screening of all siblings of onset is slow
sufferers; treatment is required for all
homozygotes, even if asymptomatic Surgical/other
(but not for heterozygotes)
• Liver transplantation Indicated
Pharmacological for approximately 5% of patients
with acute liver failure as the first
• Penicillamine A copper-chelating presentation of disease; outcomes
agent that is often used 1st line; major are usually excellent
side effects include skin disorders, • Deep brain stimulation May
nephrotic syndrome, lupus-like be effective in treating medically
systemic inflammatory conditions refractory residual neurological
and bone marrow suppression; symptoms in a subgroup of Fig. 1 Kayser–Fleischer rings, a pathognomonic sign of Wilson’s disease
patients
112 / Chapter 3 Gastroenterology: Wilson’s disease Mind Maps for Medicine \ 113
 Chapter 4
04
Renal

Acute kidney injury..................................................................................................................................................116

Chronic kidney disease..........................................................................................................................................118
Nephritic syndrome.................................................................................................................................................122
Nephrotic syndrome...............................................................................................................................................126
Urinary tract infection.............................................................................................................................................128

Mind Maps for Medicine \ 115

 • ≥65 years Pre-renal (most common)
• Stage 1 Creatinine rise of 1.5–1.9× • History of AKI
Acute kidney injury (AKI) is the abrupt baseline within 7 days or urine output • Chronic kidney disease • Hypovolaemia e.g. Haemorrhage, gastrointestinal losses, renal losses, burns, diuretic use
loss of kidney function, resulting in the <0.5ml/kg/h for >6h consecutively • Symptoms or history of urological obstruction • ↓Cardiac output e.g. Cardiac failure, liver failure, sepsis, drugs
retention of urea and other nitrogenous • Stage 2 Creatinine rise of 2.0–2.9× or conditions which may lead to obstruction • Drugs that reduce blood pressure, circulating volume, or renal blood flow (e.g. ACE
waste products and in the dysregulation baseline within 7 days or <0.5ml/kg/h • Chronic conditions e.g. heart failure, liver inhibitors, ARBs, NSAIDs, loop diuretics)
of extracellular volume and electrolytes. for >12h disease and diabetes mellitus
This can occur in the setting of previously • Stage 3 Creatinine rise of more than • Neurological or cognitive impairment Renal
normal renal function or in patients 3× baseline within 7 days or <0.3ml/kg/h or disability
with pre-existing renal disease (acute for >24h or anuria for 12h • Sepsis • Drugs e.g. ACE inhibitors, NSAIDs, aminoglycosides e.g. gentamicin, cytotoxic drugs e.g.
on chronic kidney disease). It is usually • Hypovolaemia cisplastin
characterised by a rise in creatinine. • Oliguria • Vascular e.g. Vasculitis, thrombosis, athero/thromboembolism, dissection
Diagnostic criteria • Nephrotoxic drug use within the last week • Glomerular e.g. Glomerulonephritis, Goodpasture syndrome
• Exposure to iodinated contrast agents within • Tubular e.g. Ischaemia, rhabdomyolysis, myeloma, contrast-agent induced
• The presentation will depend on the
Definition the past week • Interstitial e.g. Interstitial nephritis, e.g. ascending urinary tract infection
underlying cause and severity of AKI
Post-renal (least common) • Usually accompanied by oliguria
Risk factors or anuria
• Obstruction e.g. Renal stones, pyonephrosis, blocked catheter, pelvic mass, enlarged • Nausea, vomiting
prostate, cervical carcinoma, retroperitoneal fibrosis • Dehydration
• Confusion

Causes

Acute kidney injury Clinical features

Investigations Notes
Acute kidney injury

Urine

• Dipstick For blood, protein, leucocytes, nitrites and

Management glucose in all patients; detects treatable conditions such
as glomerulonephritis, acute pyelonephritis and interstitial
Complications nephritis
• Microscopy, culture and sensitivity
Identify cause and treat Dialysis indications (AEIOU2):
• Electrolytes and osmolality
• Pre-renal: IV fluids, treat sepsis • Acidosis (metabolic): pH <7.2 or BE <10 • Pulmonary oedema
• Hyperkalaemia Bloods
• Renal: stop nephrotoxic drugs e.g. • Electrolyte disturbance: persistent
NSAIDs, ACE inhibitors hyperkalaemia (K+ >7.0mmol/L) • Other electrolyte disturbances,
e.g. hyperphosphataemia, • FBC ↑Eosinophils in acute interstitial nephritis, cholesterol
• Intoxication with drugs e.g. lithium and embolisation and vasculitis; ↓platelets and haemolytic anaemia
Monitoring salicylates hyponatraemia, hypermagnesaemia,
hypocalcaemia suggest thrombotic microangiopathy
• Oedema-refractory pulmonary oedema • U&Es ↑Urea, creatinine ± ↑K+
• Vital signs and fluid balance • Uraemic pericarditis • Metabolic acidosis
hourly • Uraemic pericarditis • LFTs, CK ↑In rhabdomyolysis
• Uraemic encephalopathy • CRP/ESR ↑In infection/inflammation
• Daily U&Es
• Immunology ANA (SLE), ANCA (Wegener’s granulomatosis),
Referral to nephrology:
Treat complications anti-DsDNA (SLE), anti-GBM, ASOT (post-streptococcal
If the criteria for dialysis are met as above, glomerulonephritis), ↓C3, C4 (SLE)
• Treat hyperkalaemia: calcium uncertainty about cause, management • Virology Hepatitis B and C, HIV
gluconate, insulin/dextrose, or prognosis, likely diagnosis will need • ABG Detect electrolyte disturbance and metabolic acidosis
salbutamol specialist treatment (glomerulonephritis, • Blood culture For sepsis
• Treat pulmonary oedema vasculitis, tubulo-interstitial nephritis,
• Treat metabolic acidosis Imaging
myeloma), inadequate response
to treatment, or complications, history • CXR To rule out pulmonary oedema as a complication of AKI
of renal transplant or CKD stage 4 or 5, • US KUB If no identified cause of AKI or patient at risk of urinary
stage 3 AKI tract obstruction, offer urgent ultrasound of the urinary tract
• Doppler US Assessment of renal artery and veins for possible
occlusion
• MRA For more accurate assessment of renal vascular occlusion

116 / Chapter 4 Renal: Acute kidney injury Mind Maps for Medicine \ 117
 • Diabetes Bloods
• Hypertension Notes
Chronic kidney disease
• Glomerulonephritis • U&Es ↑Creatinine, ↑urea ↑K +

• Obstructive uropathy • eGFR Impaired (see Notes)

• Drugs e.g. lithium, ciclosporin, mesalazine, • FBC Normocytic anaemia (↓erythropoietin production)
aminoglycosides • Phosphate ↑ (phosphate retention)
• Polycystic kidney disease • Vitamin D ↓ Due to impaired production of
• Alport syndrome 1,25-dihydroxyvitamin D
• Recurrent kidney stones • Calcium Initially ↓ due to vitamin D deficiency but may be →
• Acute pyelonephritis/tubulointerstitial disease or ↑ if 2° and 3° hyperparathyroidism develop
Chronic kidney disease (CKD) is • SLE • Bicarbonate ↓ (metabolic acidosis)
an abnormality of kidney function or • Myeloma • PTH ↑ In 2° and 3° hyperparathyroidism
structure that is present for more than • Vasculitis • Lipid profile Dyslipidaemia is common
3 months, with implications for health. • Autoimmune screen ANA (SLE), ANCA (Wegener’s), anti-GBM
(Goodpasture syndrome)
Causes
Urine
Definition
• Urinalysis Haematuria and/or proteinuria
• Urine ACR See Notes

Imaging

• Renal US Renal size generally small in chronic renal failure;

exclude presence of obstruction/hydronephrosis; kidney stones
• AXR May reveal calcium-containing kidney stones
Classification (see Notes) Chronic kidney disease • Bone X-ray May reveal renal osteodystrophy
• Abdominal CT May reveal kidney stones, renal masses or cysts
• Abdominal MRI May reveal mass lesions in the kidney

Renal biopsy

Helps to determine pathological diagnosis of CKD if diagnosis

uncertain
Complications
Investigations
• Cardiovascular disease
• Renal anaemia Management
• Renal bone disease
• Malnutrition
• Neuropathy Renoprotection Correction of complications
• Hypertension
• Metabolic acidosis • Optimum control of BP <130/80mmHg • Metabolic acidosis → Sodium bicarbonate Clinical features
• Carpal tunnel syndrome • ACE inhibitor or angiotensin 2 receptor • Hyperphosphataemia → Dietary phosphate
• Lipid abnormalities blocker restriction and phosphate binders
• ↑Risk of infections • Anaemia → Treat underlying cause e.g. iron • Anaemia Pallor, lethargy, breathlessness
Reduce cardiovascular risk deficiency, recombinant human erythropoietin • Skin Pigmentation, pruritus
• Hyperkalaemia → Dietary restriction, diuretic • GI tract Anorexia, nausea, vomiting
• Optimum control of BP use e.g. furosemide • Endocrine Amenorrhoea, erectile
• Statins • Infection risk → Influenza and pneumococcal dysfunction, infertility
• Smoking cessation vaccinations • Neurology Confusion, coma, fits (severe
• Optimise diabetes control • Vitamin D deficiency → Vitamin D supplementation uraemia), polyneuropathy
• Low-salt diet • Cardiovascular system Hypertension,
Renal replacement therapy (see Notes) uraemic pericarditis, peripheral vascular
disease, heart failure
• Haemodialysis • Renal Nocturia, polyuria, oedema
• Peritoneal dialysis • Bone Osteomalacia, muscle weakness, bone
• Renal transplant pain, osteosclerosis, hyperparathyroidism
• Platelet abnormalities Epistaxis, bruising

118 / Chapter 4 Renal: Chronic kidney disease Mind Maps for Medicine \ 119
 Chronic kidney disease notes

Classification of CKD AKI vs CKD Haemodialysis • Haemodialysis involves pumping blood from the body through an artificial kidney in which the blood
is surrounded by a solution of electrolytes (the dialysate); solutes present in the blood at excessive
• CKD is classified based on the estimated glomerular filtration rate (eGFR) concentrations, e.g. urea, potassium, creatinine, diffuse into the dialysate and are removed; blood is drawn from
and the level of proteinuria; classifying helps to risk stratify patients AKI CKD an arteriovenous fistula and then circulated through the dialyser and returned into the fistula
• Patients are classified as G1–G5, based on the eGFR, and A1–A3 based • Heparin is constantly infused to prevent contact of blood with foreign surfaces activating the clotting cascade
on the ACR (albumin : creatinine ratio); e.g. a person with an eGFR Short duration of symptoms Long duration of symptoms • Ultrafiltration is used to regulate the distribution of water between the blood and dialysate
of 30ml/min/1.73m2 and an ACR of 15mg/mmol has CKD G3bA2 as • Haemodialysis requires the patient to have very good vascular access, which is attained by creating a fistula
detailed below: Sharp decline in renal function Gradual decline in renal function between a peripheral artery and vein (commonly radial or brachial), or a permanent catheter inserted into an
• It is important to note that patients with an eGFR of >60ml/min/1.73m2 internal jugular or subclavian vein; the fistula takes several weeks to mature and should ideally be fashioned
should not be classified as having CKD unless they have other markers Anaemia of chronic disease Anaemia of chronic disease may 3–6 months before starting haemodialysis
of kidney disease: not present be present • Haemodialysis can be carried out in a hospital setting or in the patient’s home; it is usually performed 3×/week
• persistent microalbuminuria for about 4h
• persistent proteinuria Usually normal kidney size Usually small kidney on • Complications Access-related complications (local infection, endocarditis, osteomyelitis, stenosis,
• persistent haematuria (after exclusion of other causes e.g. on ultrasound scan ultrasound scan thrombosis or aneurysm), hypotension (common), cardiac arrhythmias, air embolism, nausea / vomiting,
urological disease) headache, cramps, infected central lines, dialyser reactions, heparin-induced thrombocytopenia, haemolysis,
• structural abnormalities of the kidneys demonstrated on US or other Renal osteodystrophy not Renal osteodystrophy may be disequilibration syndrome (restlessness, headache, tremors, fits and coma), depression
radiological tests e.g. polycystic kidney disease present present
• reflux nephropathy
• biopsy-proven chronic glomerulonephritis (most of these patients Peritoneal dialysis • Peritoneal dialysis should be considered as 1st choice of treatment for: children 2 years or younger, people with
will have microalbuminuria or proteinuria, and/or haematuria) Referral to nephrologist residual renal function and adults without significant associated comorbidities
• A dialysate is infused into the peritoneal cavity and the blood flowing through peritoneal capillaries acts as the
• Advanced chronic kidney disease (category G4 or G5) blood source; ultrafiltration is controlled by altering the osmolality of the dialysate solution and thus drawing
• Rapidly deteriorating renal function water out of the patient’s blood; a permanent tube (Tenkoff catheter) is inserted into the patient’s peritoneum
• High levels of proteinuria (under local or general anaesthetic) through which dialysate is infused; the waste solutes are removed by
• Proteinuria and haematuria exchanging the peritoneal fluid for a fresh solution
• Poorly controlled HTN, despite being on 4 or more agents • The main advantage of peritoneal dialysis is that it can be performed at home, at work or while on holiday,
• Suspected rare or genetic cause of CKD therefore allows a high degree of independence and control although a great deal of support is still required
• Suspected renal artery stenosis • Complications Peritonitis, sclerosing peritonitis, catheter problems (infection, blockage, kinking, leaks or slow
drainage), constipation, fluid retention, hyperglycaemia, weight gain, hernias (incisional, inguinal, umbilical),
back pain, malnutrition and depression
Classification of chronic kidney disease using GFR and ACR categories

ACR categories (mg/mmol), description and range

Renal transplant • A kidney transplant provides the best long-term outcome for patients with end-stage kidney disease; the
kidney may come from a cadaveric donor (85–90%) or from a living donor
<3 Normal to mildly 3–30 Moderately >30 Severely
• All patients with end-stage kidney disease should be considered for a transplant; age is not a major factor itself
increased increased increased
for outcome but the presence of comorbidities significantly affects survival
• To prevent rejection, the recipients receive induction at the time of transplant with monoclonal or polyclonal
GFR and ACR categories and risk of adverse outcomes A1 A2 A3
antibodies; maintenance immunosuppression is then required in the long term to prevent rejection
• Patients need to be followed up for life and this includes annual screening for cancers, drug toxicity and
≥90 Normal and high G1
cardiovascular disease
No CKD in the absence of markers
GFR categories (ml/min/1.73m2),

• Benefits of transplantation: Can stop dialysis, improved quality of life with normal diet and activity, relaxation
60–89 Mild reduction related to normal G2 of kidney damage
of fluid restriction, reversal of anaemia and renal bone disease
description and range

range for a young adult

• Complications Postoperative problems e.g. deep vein thrombosis, pulmonary embolism and pneumonia;
Increasing risk

opportunistic infections: viral (especially herpes simplex and CMV), fungal and bacterial; malignancies
45–59 Mild–moderate reduction G3a
(particularly lymphomas and skin cancers), drug toxicity, bone marrow suppression, recurrence of the original
disease in the transplant, urinary tract obstruction, cardiovascular disease, hypertension, dyslipidaemia, graft
30–44 Moderate–severe reduction G3b
rejection (hyperacute, accelerated, acute or chronic)
15–29 Severe reduction G4

<15 Kidney failure G5

120 / Chapter 4 Renal: Chronic kidney disease notes Mind Maps for Medicine \ 121
 • Nephritic syndrome occurs as a result • Acute glomerulonephritis When nephritic Common causes in children/adolescents • Haemolytic uraemic syndrome Most commonly
of inflammatory damage to the renal syndrome occurs acutely (most common form) associated with E. coli O157:H7 and causes a triad of
endothelium • Rapidly progressive glomerulonephritis • Post-streptococcal glomerulonephritis (see Notes) microangiopathic haemolytic anaemia, thrombocytopenia
• The main causes of glomerulonephritis (RPGN) When renal function (as measured by • IgA nephropathy (see Notes) and acute kidney injury
Nephritic syndrome refers to a
share intraglomerular inflammation and the glomerular filtration rate, GFR) deteriorates • Henoch–Schönlein purpura (HSP) An IgA-mediated
group of clinical features that are Common causes in adults
include vasculitis, antibody-mediated over a period of days to weeks; it is typically small vessel vasculitis usually seen in children following
caused by acute inflammation of the
damage and immune-complex disease associated with crescents on renal biopsy; an infection; presents with palpable purpuric rash
glomeruli (glomerulonephritis). • Non-streptococcal post-infectious glomerulonephritis
underlying causes include Goodpasture (over buttocks and extensor surfaces of arms and legs,
It is characterised by haematuria, Viruses (e.g. HIV, hepatitis B and C, mumps), bacteria
syndrome, SLE and granulomatosis with see Fig. 1), abdominal pain, polyarthritis and features
proteinuria, rise in serum creatinine (e.g. staphylococci, legionella), fungi (e.g. candida and
polyangiitis of IgA nephropathy
and systemic hypertension. histoplasma), parasites (e.g. malaria and schistosomiasis)
• Chronic glomerulonephritis Nephritic
syndrome occurring over months to years • Goodpasture syndrome A rare autoimmune
with no change in the renal function disease caused by a type II antigen–antibody reaction
Definition Pathophysiology leading to diffuse pulmonary haemorrhage and
glomerulonephritis; there are circulating antiglomerular
Classification basement membrane (anti-GBM) antibodies
• Membranoproliferative glomerulonephritis
• Granulomatosis with polyangiitis (see Notes)
• Infective endocarditis
• SLE
• Cryoglobulinaemia

Fig. 1 Rash in Henoch–Schönlein purpura

Nephritic syndrome Causes

Notes
Investigations Acute respiratoryNephritic
distress syndrome

Bloods

• U&Es, eGFR Clinical features (PHAROH)

• Serum antistreptolysin-O titre
• Glucose
Management • Autoantibodies ANA, anti-DsDNA and anti-Smith antibody (SLE), anti-GBM
antibody (Goodpasture syndrome), ANCA (granulomatosis with polyangiitis) • Proteinuria (subnephrotic syndrome)
• Complement levels (C3, C4) Usually low and Pyuria
• Hepatitis and HIV screen • Haematuria: visible or non-visible
• Treat underlying cause • Azotaemia: elevated urea and
• Cryoglobulins Raised in cryoglobulinaemia
• Inflammatory renal disorders should be treated creatinine
with immunosuppressive agents, which typically Urine • Red cell casts: seen on urine
include immunosuppression with steroids, mTOR microscopy
inhibitors (e.g. sirolimus) and cytotoxic drugs e.g. • Dipstick Positive for protein and blood • Oedema and Oliguria
cyclophosphamide • Microscopy Red cell casts (see Fig. 2) • Hypertension
• Nephritic syndrome due to preformed antibodies may • Microalbumin Raised but not in
benefit from having those antibodies removed by nephrotic range
plasmapheresis, especially in the setting of pulmonary Fig. 2 Red cell cast on urine microscopy
haemorrhage Imaging
• Monitor fluid balance, weight, blood pressure and renal
function • CXR May show cavities in granulomatosis with polyangiitis or malignancy
• Restrict Na+ and K+ as appropriate • US kidneys To assess renal size and to look for renal vein thrombosis
• Fluid restriction
Renal biopsy
• Treat hypertension accordingly
• Consider prophylactic penicillin Confirms diagnosis of glomerulonephritis
• Consider dialysis for end-stage renal failure
Other

Throat swab or skin swab for Streptococcus spp. if clinically indicated

122 / Chapter 4 Renal: Nephritic syndrome Mind Maps for Medicine \ 123
 Nephritic syndrome notes Notes
Nephritic syndrome

Post-streptococcal glomerulonephritis IgA nephropathy Granulomatosis with polyangiitis

Post-streptococcal glomerulonephritis typically occurs 7–14 days following a IgA nephropathy (also known as Berger’s disease) is the commonest cause Granulomatosis with polyangiitis (previously known as Wegener’s
group A beta-haemolytic Streptococcus spp. infection (usually S. pyogenes of glomerulonephritis worldwide. It classically presents as macroscopic granulomatosis) is a rare autoimmune condition associated with a necrotising
causing a throat infection or skin infection). haematuria in young people following an upper respiratory tract infection. granulomatous vasculitis, affecting both upper and lower respiratory tract as
well as causing acute glomerulonephritis.
Pathophysiology Pathophysiology
Pathophysiology
It is caused by immune complex (IgG, IgM and C3) deposition in the glomeruli • Thought to be caused by mesangial deposition of IgA immune complexes
• There is considerable pathological overlap with HSP • There is an upregulation in the production of cytoplasmic anti-neutrophil
Clinical features cytoplasmic antibodies (c-ANCA)
Clinical features • The c-ANCA autoantibodies interact with activated neutrophils and
• Young children are most commonly affected endothelial cells within blood vessel walls, causing vessel inflammation
• General: headache, malaise, fever, nausea, anorexia • Young males more commonly affected and damage
• Haematuria • Causes recurrent episodes of macroscopic haematuria • This causes granulomatous inflammation within blood vessels, especially in
• Oedema • Typically associated with a recent upper respiratory tract infection the respiratory tract and the kidneys
• Oliguria • Nephrotic range of proteinuria is rare
• Proteinuria • Renal failure is unusual and seen in a minority of patients Clinical features
• Hypertension
Diagnostic features • Upper respiratory tract: epistaxis, sinusitis, nasal crusting
Diagnostic features • Lower respiratory tract: dyspnoea, haemoptysis
• Plasma levels of IgA are raised in about half of cases • Saddle-shape nose deformity
• Low complement levels • Histology shows mesangial hypercellularity, segmental glomerulosclerosis, • Other features: vasculitic rash, eye involvement (e.g. proptosis) and cranial
• Raised ASO titre endocapillary hypercellularity and positive immunofluorescence for nerve lesions
• Renal biopsy features: diffuse proliferative glomerulonephritis, endothelial IgA and C3
proliferation with neutrophils, subepithelial ‘humps’ caused by lumpy Diagnostic features
immune-complex deposits, immunofluorescence: granular or ‘starry sky’ Management
appearance • c-ANCA (positive in >90%), p-ANCA (positive in 25%)
• Monitor renal function and blood pressure • CXR: wide variety of presentations, including cavitating lesions
Management • Treat hypertension with ACE inhibitors or ARBs • Renal biopsy: epithelial crescents in Bowman’s capsule
• Steroids reduce the progression of kidney disease
• Treatment is usually supportive and focuses on managing hypertension • Poor prognostic indicators include hypertension and heavy proteinuria Management
and oedema
• Patients should receive penicillin to eradicate the bacteria • Treatment options include steroids, cyclophosphamide and plasma
• Carries a good prognosis exchange
• Median survival is approximately 8–9 years

124 / Chapter 4 Renal: Nephritic syndrome notes Mind Maps for Medicine \ 125
 Symptoms
Primary glomerular diseases Secondary glomerular diseases
• Oedema
• Minimal change glomerular disease • Infection e.g. HIV, hepatitis B and C, mycoplasma, • Tiredness
(accounts for 75% of cases in children and 25% syphilis, malaria • Frothiness of urine
Nephrotic syndrome is a clinical • Breathlessness (related to pleural
syndrome showing specific in adults) • Collagen vascular diseases e.g. SLE, RA, polyarteritis
• Focal segmental glomerulosclerosis: the nodosa effusion)
features of:
• PrOteinuria (≥3.5g/day) causing most common cause of idiopathic nephrotic • Metabolic diseases e.g. diabetes mellitus, amyloidosis
Signs
• HypOalbuminaemia (serum syndrome in adults • Inherited disease e.g. Alport syndrome, hereditary
albumin ≤30g/L) causing • Membranous glomerular disease nephritis • Oedema: periorbital oedema (see Fig. 1)
• Membranoproliferative glomerulonephritis • Malignant disease e.g. myeloma, leukaemia, Fig. 2 Leuconychia, which can be caused by
• Oedema (commonly in children), lower limb
lymphoma, carcinoma Fig. 1 Periorbital oedema hypoalbuminaemia
It is caused by increased oedema, oedema of genitals, ascites
permeability of serum protein in the • Drugs e.g. NSAIDs, lithium, gold, penicillamine and • Leuconychia (see Fig. 2)
renal glomerulus. Causes toxins (e.g. bee sting, snake bite) • Signs of fluid overload e.g. oedema,
• Pregnancy e.g. pre-eclampsia raised JVP
• Signs of pleural effusion e.g. dyspnoea,
stony dull percussion of chest, reduced
Definition breath sounds at bases
• Signs of dyslipidaemia: eruptive
xanthomata, xanthelasma (see Fig. 3)

• Venous thrombosis
• Sepsis Clinical features Fig. 3 Xanthelasma: yellow fat deposits underneath the skin,
• Acute kidney infection usually on or around the eyelids
• Chronic kidney disease
•
•
Hyperlipidaemia
Hypertension
Nephrotic syndrome

Complications
Notes
Nephrotic syndrome

Management
Investigations
Lifestyle Treat complications
• Urine Dipstick analysis, Bence Jones protein,
• Fluid and salt restriction • Treat fluid overload: diuretics
albumin : creatinine ratio – to quantify
• Smoking cessation • Treat proteinuria: ACE inhibitors/
proteinuria
• Exercise and balanced diet with angiotensin 2 receptor blockers
• Bloods FBC, coagulation screen, U&Es, ESR/
adequate calorific intake and sufficient • Treat hyperlipidaemia: statins
CRP, fasting glucose, immunoglobulins,
protein content (1–2g/kg daily) • Patients with severely low albumin
serum electrophoresis, autoimmune screen,
• Monitor weight regularly to help levels may require admission to
hepatitis B and C and HIV serology, lipids
determine fluid status receive IV albumin therapy
• CXR To check for pulmonary oedema/pleural
• Infection prophylaxis: influenza and
Treat underlying cause effusion
pneumococcal vaccinations
• US abdomen/kidneys To check for ascites,
• Thrombosis risk: avoid prolonged
• Immunotherapy regimen e.g. the presence of two kidneys, the size and
bed rest, consider prophylactic
prednisolone and cyclophosphamide shape of the kidneys and for any urinary tract
anticoagulation
• Stop offending drugs obstruction
• Treat hypertension
• Treat underlying cancer • Renal biopsy Under ultrasound to guide
diagnosis

126 / Chapter 4 Renal: Nephrotic syndrome Mind Maps for Medicine \ 127
 • Lower UTI Generally considered infection of the bladder (cystitis) • Female (due to shorter urethra and • Entry of bacteria into the urinary tract may be:
• Upper UTI Includes pyelitis (infection of the proximal part of the ureters) proximity to anus) • Retrograde, with ascension through urethra into
and pyelonephritis (infection of the kidneys and the proximal part of the ureters) • Increasing age bladder most commonly from faecal origin
• Recurrent UTI May be due to relapse (recurrent UTI with the same strain of organism) • Recent instrumentation of the renal tract • Via the bloodstream; is more likely in people who are
or re-infection (recurrent UTI with a different strain or species of organism) • Abnormality of the renal tract immunosuppressed
• Uncomplicated UTI Infection of the urinary tract by a usual pathogen in a person • Incomplete bladder emptying • E. coli (80%) • Direct, e.g. insertion of a catheter into the bladder,
with a normal urinary tract and with normal kidney function • Sexual activity • P. mirabilis instrumentation or surgery
• Complicated UTI Where anatomical, functional or pharmacological factors • New sexual partner • Klebsiella spp. • The urinary system has defences to prevent UTI such
predispose the person to persistent infection, recurrent infection or treatment failure • Use of spermicide • Enterococci as micturition, secreted factors and mucosal defences
e.g. abnormal urinary tract • Diabetes • Enterobacter spp. but when these defences are overcome by bacterial
• Urethral syndrome or painful bladder syndrome Symptoms of cystitis in the • Catheterisation • Staphylococcus saprophyticus: virulence factors then the patient is prone to developing
Urinary tract infection (UTI) is absence of UTI; this syndrome is also called interstitial cystitis, bladder pain syndrome • Institutionalisation the 2nd leading cause in sexually a UTI
presence of characteristic symptoms and trigonitis • Pregnancy active females • Virulence factors include fimbriae which allow binding,
and significant bacteriuria from • Immunocompromised/ • Pseudomonas aeruginosa and a bacterial capsule that resists phagocytosis
kidneys to bladder. immunosuppressed • Candida albicans (rare) (uropathogenic Escherichia coli); Proteus mirabilis
Classification produces urease and increases the pH of urine

Definition Risk factors Causes

Pathophysiology

Notes
Complications Urinary tract infection Acute respiratory
Urinary
distress
tractsyndrome
infection

• Pyelonephritis
• Perinephric and intrarenal abscess
• Hydronephrosis or pyonephrosis
• Acute kidney injury
• Sepsis

Management Investigations Clinical features

Conservative • Urine dipstick Often positive • Urinary frequency

for nitrite and/or leucocytes, may • Dysuria
• Education about the condition and avoidance of certain risk factors e.g. spermicide use show microscopic haematuria • Haematuria
• Vaginal oestrogen therapy in post-menopausal women should be considered as a preventative • Urine microscopy, culture • Foul-smelling ± cloudy urine
measure and sensitivity Shows high • Urgency
• There is no significant evidence that drinking cranberry juice, increasing fluid intake or personal WCC, causative organism and • Urinary incontinence
hygiene measures makes a difference sensitivities to antibiotics • Suprapubic pain and tenderness
• d-mannose can be used to prevent UTIs • US/CT KUB Should be considered • Loin/flank pain and tenderness (suggest upper UTI)
to rule out urinary obstruction/ • Rigors
Pharmacological collection in acute uncomplicated • Pyrexia
pyelonephritis or to rule out • Nausea ± vomiting
• Empirical treatment: trimethoprim or nitrofurantoin for 3 days – the drugs of choice for the
structural causes for recurrent UTIs • Acute confusional state (delirium): particularly elderly patient
empirical treatment of uncomplicated lower UTI
or complicated UTIs
• Oral ciprofloxacin for 7–10 days or co-amoxiclav or cefalexin are choices for upper UTI
• Pregnant women with symptomatic bacteriuria should be treated with an antibiotic for 7 days;
a urine culture should be sent
• Men, patients with ‘complicated UTI’ and catheterised patients should be treated for 7 days
• Prophylactic antibiotics with low-dose trimethoprim or nitrofurantoin can be used
for recurrent cystitis
• Paracetamol and/or NSAIDs can be used for symptomatic treatment

128 / Chapter 4 Renal: Urinary tract infection Mind Maps for Medicine \ 129
 Chapter 5
05
Endocrinology

Acromegaly...................................................................................................................................................................132
Adrenal insufficiency...............................................................................................................................................134
Cushing syndrome...................................................................................................................................................136
Diabetes insipidus.....................................................................................................................................................138
Diabetes mellitus: overview and management.....................................................................................140
Diabetic ketoacidosis..............................................................................................................................................148
Hyperaldosteronism................................................................................................................................................152
Hypocalcaemia...........................................................................................................................................................154
Hypercalcaemia and hyperparathyroidism...............................................................................................156
Hyperprolactinaemia..............................................................................................................................................158
Hypothyroidism..........................................................................................................................................................160
Hyperthyroidism........................................................................................................................................................164
Hypoglycaemia...........................................................................................................................................................168
Hyponatraemia...........................................................................................................................................................170
Hypopituitarism.........................................................................................................................................................174
Phaeochromocytoma.............................................................................................................................................176
Polycystic ovary syndrome..................................................................................................................................178

Mind Maps for Medicine \ 131

 • Under the influence of GHRH and somatostatin (SST), • Headache Common and often
both produced by the hypothalamus, GH is secreted – Hypothalamus + unrelated to size of adenoma
in a pulsatile manner • Facial changes (see Figs. 2 and 3)
SST GHRH
• Hypoglycaemia stimulates GH secretion while • Skin changes Include excess
hyperglycaemia has the opposite effect sweating and skin tags
• GHRH stimulates GH production by the somatotrophs Pituitary • Sexual dysfunction Subfertility,
Acromegaly is a rare disorder caused of the anterior pituitary gland, and somatostatin-14 GH
amenorrhoea, loss of libido
by excessive secretion of growth inhibits both GH and thyroid-stimulating hormone • Hands and feet These grow
FFA Stomach Ghrelin (see Fig. 4) leading to difficulty
hormone (GH) and it is almost (TSH) secretion
• Ghrelin hormone produced by the GI tract acts on in wearing rings and increase in Fig. 2 Characteristic facial features of acromegaly including
always due to a benign pituitary Adipose tissues
the hypothalamus to stimulate GHRH and also acts shoe size coarsening of the general appearance, widening of the nose, Fig. 4 The hand of a patient with acromegaly: ‘spade like’ (left)
adenoma. It is very rarely caused IGF-1 Stress
on the anterior pituitary gland directly to promote • Visual field defects (see Fig. 5) thicker lips, development of frontal bossing and prognathism compared with someone with normal-sized hands
by ectopic production of GH or Amino acids
Liver • Joint pain Common, mainly load-
unregulated GH-releasing hormone GH secretion hypoglycaemia
(GHRH) secretion. It results in an • GH acts on adipose tissue (to promote lipolysis), on the bearing joints and kyphoscoliosis Left Right
liver (to promote gluconeogenesis and stimulate the Fig. 1 Control of GH secretion; FFA, free fatty acids • Fatigue
overgrowth of all organ systems,
bones, joints and soft tissues. release of insulin-like growth factor 1, IGF-1) and on • Carpal tunnel syndrome
muscle (to promote protein synthesis) • Galactorrhoea
• IGF-1 itself ↑somatic cell growth, chondrocyte function and bone modelling/remodelling
Definition • The negative feedback effects on the control of GH secretion are via IGF-1 and by feedback
of GH on the hypothalamus (see Fig. 1)
Clinical features Fig. 5 The most common defect is a bitemporal hemianopia
caused by compression of the optic chiasm by the pituitary

Growth hormone physiology

Fig. 3 Macroglossia seen in a patient with acromegaly adenoma

• Pituitary adenoma (95% of cases)

• Ectopic GH from non-endocrine tumours (rare) e.g. lung cancer, pancreatic
cancer, ovarian cancer
Acromegaly Causes • Familial: as part of MEN-1, familial acromegaly, Carney’s complex or
McCune–Albright syndrome

Notes
Acromegaly

Complications Management Investigations

• Impaired glucose tolerance or Pharmacological • Blood: glucose, serum phosphate,

diabetes urinary calcium and serum triglycerides
• Cardiovascular disease: • SST analogues 1st-choice medical treatment, (may all be raised)
hypertension, ischaemic heart e.g. octreotide • IGF-1: used for initial screening as it is
disease, cardiomyopathy, heart • Dopamine agonists Effective highly sensitive (normal level usually
failure (e.g. cabergoline) but less so than excludes acromegaly)
• Colonic polyps and SST analogues • Oral glucose tolerance test: if the
adenocarcinoma of the colon • GH receptor antagonists e.g. Pegvisomant glucose load fails to suppress GH it is
• Obstructive sleep apnoea is licensed for the treatment of acromegaly in diagnostic of acromegaly
• Hyperprolactinaemia, patients with inadequate response to surgery, • Prolactin, adrenal, thyroid and gonadal
↓glucocorticoids, sex steroids and radiotherapy or somatostatin analogues hormones (tumour may cause
thyroid hormone hypopituitarism)
• Hypopituitarism (post-surgery Surgical • Visual field assessment: the most
or radiotherapy) common defect is a bitemporal
• Trans-sphenoidal surgery Treatment of
hemianopia due to compression of the
choice in most cases
optic chiasm
Radiotherapy • MRI of the pituitary gland and
hypothalamus to confirm the pituitary
• Used for refractory disease, as an adjuvant for tumour
large invasive tumours and when surgery is • Cardiac assessment: ECG, ECHO (to rule
contraindicated out cardiovascular complications)

132 / Chapter 5 Endocrinology: Acromegaly Mind Maps for Medicine \ 133

 • Adrenal insufficiency May be primary, thyroid disease, type 1 diabetes and Primary adrenal insufficiency Acute (may present as a ‘crisis’)
secondary or tertiary pernicious anaemia
• Primary adrenal insufficiency The adrenal • Tuberculosis The leading cause of primary • Autoimmunity (Addison’s disease): accounts for approx. 85% of • Hypotension
gland cortex is destroyed therefore it has adrenal insufficiency worldwide and is cases of primary adrenal insufficiency in the developed world • Hypovolaemic shock
impaired ability to secrete hormones often in combination with HIV infection • Infections: TB (most common cause of primary adrenal insufficiency • Acute abdominal pain
(but hypothalamus and pituitary gland • Secondary adrenal insufficiency Results world-wide), HIV, histoplasmosis, cryptococcosis, syphilis • Low-grade fever and vomiting
function remain intact); this leads to loss of from disruption of adrenocorticotropic • Trauma • Collapse
both cortisol and aldosterone secretion hormone (ACTH) secretion by the pituitary • Post adrenalectomy
• Addison’s disease Most common cause gland (hypopituitarism) and tertiary • Invasion: e.g. neoplastic, sarcoidosis, amyloidosis, haemochromatosis Chronic (‘develops insidiously and may be mild’)
Adrenal insufficiency is a group of primary adrenal insufficiency in the adrenal insufficiency results from loss
of disorders in which there is West; there is progressive destruction of CRH secretion due to hypothalamic Secondary adrenal insufficiency Symptoms:
a failure to produce adequate of the adrenal glands via autoimmune damage or long-term glucocorticoid • Lethargy • Confusion/personality
amounts of adrenal hormones, mechanisms and antibodies against steroid treatment • Pituitary disorders: e.g. tumours, irradiation, infiltration, • Weakness change
i.e. glucocorticoids (cortisol) 21-hydroxylase can be found in about • Secondary and tertiary causes Result Sheehan syndrome • Anorexia • Diarrhoea
and/or mineralocorticoids 85% of patients in glucocorticoid but not aldosterone • Isolated ACTH deficiency • Nausea and vomiting • Constipation
(aldosterone). • Addison’s disease Associated with other deficiency as aldosterone is not under the • Chronic opiate usage • Abdominal pain • Syncope or dizziness
autoimmune conditions e.g. autoimmune control of ACTH • Weight loss • Irritability
Tertiary adrenal insufficiency
Definition • ‘Salt-craving’
• Long-term glucocorticoid treatment (accounts for approx.
Pathophysiology 99% of cases of adrenal insufficiency) Signs:
• Directly following cure of Cushing syndrome • Hyperpigmentation (primary adrenal insufficiency only, see
• Hypothalamic impairment: infiltrative disease e.g. sarcoidosis, Fig. 1): often at buccal mucosa, lips, palmar creases, new scars
tumours, irradiation (not present in secondary adrenal insufficiency)
• Hypotension
Causes • Postural hypotension

Adrenal insufficiency

Fig. 1 Hyperpigmentation in buccal mucosa in patients with

primary adrenal insufficiency
Notes
Adrenal insufficiency

Clinical features
Management

Acute (Addisonian crisis)

• Aggressive fluid resuscitation with IV normal saline

• Immediate administration of hydrocortisone e.g. 100mg Investigations
IV or IM
• Continuous cardiac and electrolyte monitoring
• Treatment of the underlying precipitating disorder,
e.g. treat infection with antibiotics Screening tests Diagnostic tests

Long-term • Bloods FBC (possibly anaemia, mild eosinophilia and • ACTH (↑ in 1° adrenal insufficiency and ↓ in 2° adrenal insufficiency)
lymphocytosis), U&Es (↓Na+, ↑K+), LFTs (possible raised • Cortisol levels (0900h) Low; ≥500nmol/L makes Addison’s very unlikely
• Hormone replacement Hydrocortisone and fludrocortisone ALT), glucose (↓), Ca2+ (possibly ↑), adrenal autoantibodies • Insulin tolerance test Hypoglycaemia is induced by an insulin infusion and
• Patient education: e.g. anti-21-hydroxylase for Addison’s the cortisol response is monitored; confirms 2° adrenal insufficiency
• Information about the condition • ABG As above and may show metabolic acidosis • ACTH stimulation (Synacthen®) test ACTH is administered IV or IM, and
• Medical emergency identification bracelet and steroid card • Imaging CXR (rule out lung cancer), CT or MRI of adrenal cortisol levels measured 30min later; the normal response is ↑ in cortisol; in
• Importance of not missing steroids/stopping them abruptly gland (investigate primary causes) or hypothalamic pituitary 1° adrenal insufficiency this does not occur
• Intercurrent illness: if tolerating oral medication then region (investigate secondary causes) • CRH test Used to differentiate between 2° and 3° adrenal insufficiency; after
double dose, and if unable to take orally seek urgent administration of CRH, ACTH response is measured. Patients with 2° adrenal
medical help insufficiency, i.e. pituitary disease, do not respond whereas those with
• Advice for travel: carry extra medication and an emergency hypothalamic disease, i.e. 3° adrenal insufficiency, do respond
self-injection kit

134 / Chapter 5 Endocrinology: Adrenal insufficiency Mind Maps for Medicine \ 135
 – ACTH dependent Signs Symptoms
Hypothalamus
• Glucocorticoids, most importantly • Cushing disease: excess ACTH from the • ‘Moon-shaped’ face (see Fig. 2) • Depression
cortisol, are produced by the adrenal pituitary (80%) • Striae (see Fig. 3) • Difficulty with weight
cortex Corticotrophin releasing • Ectopic ACTH-producing tumour: e.g. • Proximal muscle weakness management
• The release of cortisol is under the control hormone (CRH) small cell lung cancer (5–10%) • Plethoric facies • Fatigue
of corticotropin-releasing hormone • Excess exogenous ACTH • Easy bruising • Psychotic features
+
(CRH) by the hypothalamus and administration • Weight gain with poor linear growth • Decreased libido
adrenocorticotropic hormone (ACTH) (in children and adolescents) • Menstrual abnormalities
–

Negative feedback
by the anterior pituitary gland (see Fig. 1) Anterior pituitary gland ACTH independent • Buffalo hump (fat pad sign) • Back pain Fig. 2 ‘Moon-shaped’ face
• The cause of Cushing syndrome can be • Central obesity
Cushing syndrome is the term
either exogenous (most common) or • Excess exogenous glucocorticoid • Thin skin and poor healing
used to describe a range of signs
endogenous administration (most common) • Hirsutism with crown hair loss
and symptoms which occur as a
• Endogenous causes result from the ACTH • Adrenal adenoma • Acne
result of prolonged exposure to
adrenal gland itself producing excessive • Adrenal carcinoma (rare) • Virilisation, short stature, advanced
glucocorticoids.
cortisol (ACTH independent) or in + • Familial Cushing syndrome and or delayed puberty in children
response to excessive stimulation from other rare causes such as MEN-1, • ↑Pigmentation (ACTH-dependent
ACTH (ACTH dependent) produced McCune–Albright syndrome, adrenal
Definition by the anterior pituitary gland or from Adrenal cortex macronodular hyperplasia and food-
Cushing syndrome only)
Fig. 3 Abdominal striae
elsewhere induced Cushing syndrome

Cortisol Causes Clinical features

Pathophysiology Fig. 1 The hypothalamus–pituitary–adrenal axis

Cushing syndrome
Investigations
Diagnosis of Cushing syndrome involves a 2 step process: first, confirming
raised cortisol levels, followed by investigating the underlying cause (provided
an obvious cause such as exogenous corticosteroids is not present)
1. Confirm raised cortisol 2. Establish cause for ↑cortisol
Complications Management
• Urinary free cortisol Simple and non-invasive;
very sensitive but not specific Measure ACTH level
• ‘Steroid diabetes’ • Stop/reduce dose of offending drug for • Overnight dexamethasone suppression test
• Osteoporosis exogenous Cushing syndrome Give dexamethasone 1mg PO at midnight and
• Hypertension • Consider steroid-sparing agents such as measure serum cortisol at 0800h; in Cushing
• Coagulopathy azathioprine for exogenous Cushing syndrome syndrome, there is no cortisol suppression
• Metabolic syndrome • Trans-sphenoidal microsurgery or • Low-dose dexamethasone suppression Low High
• Immunosuppression radiotherapy-adjunct for pituitary tumours test Patient is given dexamethasone 0.5mg/6h • High-dose dexamethasone suppression test >90%
• Nelson syndrome: ACTH- (Cushing disease) orally for 2 days; measure cortisol at 0 and 48h; reduction in basal urinary free cortisol levels supports
secreting tumour develops • Adrenalectomy: ‘cures’ adenomas but rarely in Cushing syndrome, there is failure to suppress pituitary adenoma diagnosis
following therapeutic total cures cancer cortisol Adrenal cause Cushing disease or ectopic • Blood gas Hypokalaemia alkalosis >95% in ectopic
bilateral adrenalectomy (TBA) • Treat underlying cancer in ectopic cortisol • Midnight salivary cortisol Reflects free plasma (adenoma or carcinoma) secretion tumour secretion, <10% in Cushing disease
for Cushing syndrome secretion cortisol since there is no cortisol-binding globulin • Inferior petrosal sinus (IPS) sampling Performed with
• Cataracts • Metyrapone, ketoconazole, and mitotane can in saliva; has good sensitivity and specificity CRH stimulation; a higher IPS to peripheral ACTH ratio
all be used to lower cortisol by directly inhibiting suggests pituitary adenoma rather than ectopic secretion
synthesis and secretion in the adrenal gland Adrenal imaging
• MRI pituitary To determine pituitary adenoma
Abdominal CT or MRI if Arrange • Full body CT scan To look for underlying malignancy
exogenous glucocorticoids further tests • Plasma CRH Ectopic CRH production is a very rare cause
are excluded as the cause of Cushing disease

136 / Chapter 5 Endocrinology: Cushing syndrome Mind Maps for Medicine \ 137
 • Increased plasma osmolality is detected by osmoreceptors in the Cranial
anterior hypothalamus which stimulates the posterior pituitary
gland to secrete ADH • Idiopathic
• ADH acts on the distal convoluted tubule and collecting duct • There are two major forms of DI: • Tumours e.g. craniopharyngioma, germinoma, hypothalamic metastases
resulting in ↑water reabsorption thus restoring plasma osmolality • Cranial DI Decreased secretion of • Head injury
(see Fig. 1) ADH resulting in reduced ability to • Granulomatous conditions e.g. sarcoidosis, TB, Wegener’s
Diabetes insipidus (DI) is a condition • ADH secretion Hypothalamus
concentrate urine granulomatosis
caused by hyposecretion of, or is suppressed • Nephrogenic DI Reduced ability • Infections e.g. encephalitis, meningitis, cerebral abscess • Polyuria
insensitivity to the effects of, when plasma to concentrate urine because of • Post radiotherapy • Polydipsia
antidiuretic hormone (ADH), also osmolality Plasma osmolality Posterior resistance to ADH in the kidney • Vascular e.g. haemorrhage/thrombosis, aneurysms, Sheehan syndrome • Nocturia
high pituitary
known as arginine vasopressin is below • DI must be distinguished from • Congenital defects in the ADH gene: DIDMOAD • Urinary incontinence (may result if there is damage
(AVP). Its deficiency or failure to act 280mOsm/kg, Kidney
primary polydipsia, which is a to the bladder through chronic overdistension)
causes an inability to concentrate allowing maximal (distal ADH psychiatric disturbance characterised Nephrogenic • Signs of dehydration
tubule)
urine in the distal renal tubules, water diuresis
Plasma osmolality by excessive intake of water, and other • Palpable bladder
Urine osmolality
leading to the passage of copious Water causes of polyuria and polydipsia e.g. • Inherited (mutations in the ADH receptor) • In infants may present with irritability, failure to thrive,
reabsorption
volumes of dilute urine. hyperglycaemia • Metabolic: hypercalcaemia and hypokalaemia protracted crying, fever, anorexia and fatigability or
• Chronic kidney disease feeding problems
Fig. 1 ADH release
• Drugs e.g. lithium, meclocycline
Definition Pathophysiology
ADH physiology Clinical features
Causes

Diabetes insipidus

Notes
Diabetes insipidus

Investigations
Management • Urine 24-h urine collection (>3L/24h), osmolality (↓osmolality, <300mOsm/kg)
• Water deprivation test The patient is deprived of fluids for up to 8h or until 5% loss
of body weight, following which desmopressin 2µg IM is given (see Table 1)
Cranial DI • Bloods Glucose/HbA1c (exclude diabetes), U&Es, plasma osmolality (↑)
• MRI Pituitary, hypothalamus and surrounding tissues
• Desmopressin (a synthetic • US KUB May be used to assess for obstructive complications caused by the high
replacement for vasopressin) to increase urinary back-pressure
water reabsorption
Table 1 Water deprivation test interpretation
• Treat any underlying infections
• Surgical excision of tumours Urine osmolality Urine osmolality Likely diagnosis
after fluid deprivation after desmopressin
Nephrogenic DI
(mOsm/kg) (mOsm/kg)
• Have access to drinking water and to
drink enough to satiate thirst >600 >600 Normal
• Correct any metabolic abnormality
• Stop any drugs that may be causing the <300 >800 Cranial DI
problem
• High-dose desmopressin may be used <300 <300 Nephrogenic DI
with success in mild-to-moderate cases
of nephrogenic DI >800 >800 Primary/psychogenic
• Thiazide diuretic and NSAIDs polydipsia (PP)

300–800 <800 Partial cranial DI or

nephrogenic DI or PP or
diuretic abuse

138 / Chapter 5 Endocrinology: Diabetes insipidus Mind Maps for Medicine \ 139
 T1DM T2DM

Genetics: • Obesity (especially central adiposity)

• Family history: about 15% of people with T1DM have a 1st-degree • Sedentary lifestyle
• Diabetes is one of the most common chronic relative with the condition • Ethnicity: South Asian, African–Caribbean, Middle Eastern at greater risk
diseases in the UK, and its prevalence is increasing • The class II gene products HLA-DR3 and -DR4 have been associated of T2DM compared with White population
• NICE estimates that by 2025, more than 5 million with increased risk of T1DM • History of gestational diabetes
Diabetes mellitus is a group of metabolic disorders
people in the UK will be diagnosed with diabetes • Impaired glucose tolerance/impaired fasting glucose
in which persistent hyperglycaemia is caused by
• T1DM can occur at any age but it most commonly • Drug therapy e.g. combined use of a thiazide diuretic with a beta
deficient insulin secretion, resistance to the action Environmental: blocker, statins
of insulin, or both: presents in children and young people
Genetically susceptible people, unknown infectious agents or components • Low-fibre, high-glycaemic index diet
• Type 1 diabetes mellitus (T1DM): an absolute insulin • Approx. 90% of those with diabetes have T2DM;
of early childhood diet can trigger the development of autoimmunity to • Metabolic syndrome
deficiency which causes persistent hyperglycaemia they are usually older at presentation (>30 years)
the beta cells in the pancreatic islets of Langerhans • Polycystic ovary syndrome
• Type 2 diabetes mellitus (T2DM): insulin resistance but it is increasingly being diagnosed in children
and adolescents due to increasing obesity • Family history (2.4-fold increased risk)
and a relative insulin deficiency resulting in persistent
• Adults who had low birth weight for gestational age
hyperglycaemia
Risk factors
Definition Epidemiology Notes
Diabetes mellitus: overview

Diabetes mellitus: overview Pathophysiology

T1DM

• The development of T1DM is based on a combination

of a genetic predisposition and an autoimmune process
that results in gradual destruction of the beta cells of the
Complications Diagnostic criteria Clinical features pancreas, leading to absolute insulin deficiency
• There is usually a pre-diabetic phase where autoimmunity
has already developed, with insulin autoantibodies being
Acute • Diabetes may be diagnosed on • Osmotic symptoms: polyuria, detected in genetically predisposed individuals as early as
the basis of 1 abnormal plasma polydipsia 6–12 months of age
• Hypoglycaemia glucose + diabetic symptoms • Lethargy • These include islet cell antibodies against cytoplasmic
• Diabetic ketoacidosis or 2 abnormal tests in an • Boils, pruritus vulvae or with frequent, proteins in the beta cells, antibodies to glutamic acid
• Hyperglycaemic hyperosmolar asymptomatic individual: recurrent or prolonged infections decarboxylase (GAD-65) and insulin autoantibodies to
state (HHS) • Fasting plasma glucose • Patients with T1DM may present protein tyrosine phosphatase
≥7.0mmol/L acutely with weight loss, dehydration, • Possible triggers for the process may include viruses, dietary
Long-term • Random plasma glucose ketonuria and hyperventilation factors, environmental toxins and emotional or physical stress
≥11.1mmol/L • Presentation in patients with T2DM
Microvascular complications: tends to be subacute with a longer T2DM
• Oral glucose tolerance
• Nephropathy test (OGTT) Plasma glucose duration of symptoms or with
• T2DM is characterised by a combination of peripheral
• Retinopathy concentration ≥11.1mmol/L 2h complications e.g. retinopathy picked
insulin resistance and inadequate insulin secretion by
• Peripheral neuropathy after 75g anhydrous glucose up on routine optician appointment
pancreatic beta cells
• Autonomic neuropathy • HbA1c ≥48mmol/mol (6.5%) or asymptomatic and picked up on
• Insulin resistance is strongly linked to obesity and physical
• Increased risk of diabetes: routine bloods
inactivity in genetically susceptible individuals
Macrovascular complications: • Impaired glucose tolerance (IGT) • In patients with T2DM, there has been found to be elevated
• Cardiovascular disease Fasting plasma venous glucose levels of free fatty acids and pro-inflammatory cytokines
e.g. myocardial infarction <7mmol/L and 2 OGTT plasma in the plasma which lead to decreased glucose transport
• Cerebrovascular disease venous glucose 7.8–11.0mmol/L into muscle cells, elevated hepatic glucose production and
e.g. stroke, TIA • Impaired fasting glucose Plasma increased breakdown of fat
• Peripheral arterial disease venous glucose of 6.1–6.9mmol/L • The beta cell mass is reduced by about a half at the time
e.g. intermittent claudication • Pre-diabetes HbA1c 42–47mmol/ of diagnosis
mol (6–6.4%)

140 / Chapter 5 Endocrinology: Diabetes mellitus: overview Mind Maps for Medicine \ 141
 Diabetes mellitus: type 1 management Notes
Diabetes mellitus: type 1 management

Education and information Modification of other risk factors Monitoring and targets
All adults with type 1 diabetes mellitus (T1DM) should be referred to Blood pressure HbA1c
a structured education programme of proven benefit e.g. the DAFNE
(dose adjustment for normal eating) programme. • If there is no albuminuria or features of the metabolic syndrome, the • Should be monitored every 3–6 months
threshold for starting antihypertensive treatment in an adult is a BP • Adults should have a target HbA1c level of 48mmol/mol (6.5%) or lower
Dietary advice ≥135/85mmHg
• If there is albuminuria, or 2 or more features of the metabolic syndrome,
• Individual factors such as the person’s daily activities, aspirations, likelihood
of complications, comorbidities, occupation and history of hypoglycaemia
• A low glycaemic index diet for blood glucose control should not be advised the threshold for starting antihypertensive treatment is a BP ≥130/80mmHg should be strongly taken into account
• Patients should be offered carbohydrate-counting training as part of the • ACE inhibitors or angiotensin-II receptor antagonists are 1st-line treatment
structured education programme for self-management for treating hypertension Self-monitoring of blood glucose
• Advise adults with T1DM on the importance of a healthy, balanced diet in
Lipids • Recommend testing at least 4 times a day, including before each meal and
reducing the risk of cardiovascular disease; the diet should be low in fat,
before bed
sugar and salt, and contain at least 5 portions of fruit and vegetables a day
• Statin treatment should be offered with atorvastatin 20mg for the primary • More frequent monitoring is recommended if frequency of hypoglycaemic
• Dietitian support may be required to help optimise body weight and blood
prevention of CVD if the person: episodes increases; during periods of illness; before, during and after sport;
glucose control
• Is older than 40 years, or when planning pregnancy, during pregnancy and while breastfeeding
• Has had diabetes for more than 10 years, or • Recommended blood glucose levels:
Exercise • Has established nephropathy, or • 5–7mmol/L on waking and
• Has other CVD risk factors (such as obesity and hypertension) • 4–7mmol/L before meals at other times of the day
• Adults with T1DM should be advised to regularly exercise (30min 5 times/
• Statin treatment with atorvastatin 80mg for the secondary prevention
week) as this lowers blood glucose levels and reduces their increased
of CVD
cardiovascular risk long term DVLA and diabetes mellitus
• They should also be advised on the effect of activity on blood glucose levels Smoking (see Ch5: Diabetes mellitus: type 2 management)
when insulin levels are adequate i.e. risk of hypoglycaemia
• The recommendations of exercise are the same as those of T2DM Patients with T1DM who smoke should be given advice on smoking cessation
(see Ch5: Diabetes mellitus: type 2 management) and appropriately referred to smoking cessation services. Sick day rules
Alcohol During a period of illness (that does not warrant admission), the patient should
Insulin and other pharmacological adhere to the following ‘sick day rules’:
agents • Patients with diabetes should drink alcohol within the safe limits • Not stop their insulin therapy
(14 units/week) • The dose of insulin may need to be altered during periods of illness; they
Insulin • Alcohol can exacerbate or prolong hypoglycaemia, or cause signs of should seek advice from their diabetes team if unsure of how to adjust
hypoglycaemia to be less clear or delayed, and therefore patients should insulin doses
• All patients with T1DM will require insulin treatment avoid drinking on an empty stomach, avoid binging and wear some form of • Monitor blood glucose levels more frequently; this should be done at least
• Almost all insulin preparations are synthetic (recombinant) human insulin diabetes identification (as the reduced awareness of hypoglycaemia may be every 3–4h including through the night, and sometimes every 1–2h
• Insulin is injected into the subcutaneous tissue of the abdomen, thighs or confused with alcohol intoxication) e.g. medic alert bracelet • Consider ketone monitoring (blood or urine); if the urine ketone level is
upper arm greater than 2+, or blood ketone levels are greater than 3mmol/L, the
• Choice of insulin regimen depends on several factors, e.g. age, duration of person should contact the GP or diabetes care team immediately
diabetes, family lifestyle, school support and socioeconomic factors Screening and managing • Maintain normal meal pattern (where possible) if appetite is reduced;
• Multiple daily injection (MDI) basal-bolus insulin is the regimen of choice complications normal meals could be replaced with carbohydrate-containing drinks
• Long-acting insulin e.g. Twice-daily insulin detemir as basal insulin therapy (such as milk, milk shakes, fruit juices and sugary drinks)
for adults with T1DM (see Ch5: Diabetes mellitus: type 2 management)
• Aim to drink at least 3L (5 pints)/day to prevent dehydration; if vomiting or
• Rapid-acting insulin Offer rapid-acting insulin analogues injected before diarrhoea is persistent, they should seek immediate medical advice as IV
meals e.g. insulin aspart for mealtime insulin replacement fluids may be required
• Mixed insulin Consider a twice-daily human mixed insulin regimen for • Seek urgent medical advice if they are violently sick, drowsy or unable to
adults with T1DM if an MDI basal-bolus insulin regimen is not possible keep fluids down
• Common complications of insulin therapy include hypoglycaemia,
lipohypertrophy (at injection site), insulin resistance and weight gain
• Continuous subcutaneous insulin infusion (or ‘insulin pump’) therapy
is recommended as a treatment option if attempts to achieve target HbA1c
levels with multiple daily injections result in the person experiencing
disabling hypoglycaemia, or if HbA1c remains high despite being on MDI
therapy

Metformin

NICE recommend considering adding metformin if BMI ≥25kg/m²

142 / Chapter 5 Endocrinology: Diabetes mellitus: type 1 management Mind Maps for Medicine \ 143
 Diabetes mellitus: type 2 management

Patient education Modification of other risk factors Screening and managing DVLA and diabetes mellitus
Patients with type 2 diabetes mellitus (T2DM) or at risk of T2DM should be Blood pressure complications
offered referral to a structured education programme such as the DESMOND Group 1 driving entitlement
Ensure the person is screened for the following at diagnosis and then at least
(Diabetes Education for Self-Management for Ongoing and Newly Diagnosed) • If <80y target clinical BP <140/90mmHg and if ≥80y target clinical BP annually: • If diet-controlled alone then no requirement to inform Driver and Vehicle
programme. <150/90mmHg • Retinopathy Patients should receive retinal examination as part of the NHS Licensing Agency (DVLA)
• ACE inhibitors or angiotensin-II receptor antagonists are 1st line in patients retinopathy screening programme • If on tablets (that do not cause hypoglycaemia) or glucagon-like peptide-1
Dietary advice with diabetes • Diabetic foot problems Check for neuropathy (including use of a 10g (GLP-1) analogue there is no need to notify DVLA
monofilament), ulcers, callus formation, infection, deformity, gangrene and • Do not need to inform DVLA if on tablets that may induce hypoglycaemia
• High-fibre diet low glycaemic index sources of carbohydrates Lipids
Charcot arthropathy; foot care advice should be given including appropriate (e.g. sulfonylureas) unless there has been more than one episode of severe
• Low-fat dairy products and oily fish footwear and for patients to regularly check their own feet; they may require hypoglycaemia (requiring the assistance of another person) within the
Patients with a 10-year cardiovascular risk >10% (using QRISK2) should be
• Reduce the intake of foods containing saturated fats and trans fatty acids referral to podiatry preceding 12 months
offered a statin; 1st-line statin of choice is atorvastatin 20mg ON for primary
• Initial target weight loss in an overweight person is 5–10% • Nephropathy Check serum U&Es and urine albumin: creatinine ratio (ACR); • If on insulin they need to inform the DVLA; the patient can drive a car as
prevention and atorvastatin 80mg ON for secondary prevention
• Discourage the use of foods marketed specifically for people with diabetes patients with nephropathy should be put on ACE inhibitor or ARB provided long as they have hypoglycaemic awareness, not more than one episode
• Consider referral to dietitian Smoking there are no contraindications of severe hypoglycaemia within the preceding 12 months and no relevant
• Cardiovascular risk factors Check smoking status, blood pressure, BMI and visual impairment
Exercise Patients with T2DM who smoke should be given advice on smoking cessation cholesterol and manage these accordingly (see above)
Group 2 driving entitlement
and appropriately referred to smoking cessation services. • Peripheral and autonomic neuropathy, erectile dysfunction and
Regular exercise may lower blood glucose levels; exercise recommendations gastroparesis: ask specifically in the history and manage accordingly
include: Alcohol The following standards need to be met for patients on insulin or other
• At least 150min/week of moderate intensity physical activity e.g. brisk hypoglycaemic drugs, e.g. sulfonylureas:
walking or cycling in bouts of ≥10min, or • Patients with diabetes should drink alcohol within the safe limits Monitoring and targets • There has not been any severe hypoglycaemic event in the previous
• 75min vigorous intensity activity (such as running or playing football) spread (14units/week) 12 months
• Alcohol can exacerbate or prolong hypoglycaemia, or cause signs of HbA1c • The driver has full hypoglycaemic awareness
across the week or combinations of moderate and vigorous intensity activity
• All adults should also undertake physical activity to improve muscle strength hypoglycaemia to be less clear or delayed; therefore patients should avoid • The driver must show adequate control of the condition by regular blood
drinking on an empty stomach, avoid binging and wear some form of • Individual targets should be agreed with patients to encourage motivation; glucose monitoring, at least twice daily and at times relevant to driving
on at least 2 days per week HbA1c targets are dependent on treatment:
• Time spent being sedentary should be minimised diabetes identification (as the reduced awareness of hypoglycaemia may be • The driver must demonstrate an understanding of the risks of
confused with alcohol intoxication) e.g. medic alert bracelet • Managed by lifestyle only 48mmol/mol (6.5%) target hypoglycaemia
• Older adults (≥65 years) who are at risk of falls should incorporate physical • Managed by lifestyle and metformin 48mmol/mol (6.5%) target
activity to improve balance and coordination on at least 2 days per week • Management includes any drug which may cause hypoglycaemia
53mmol/mol (7.0%) target Sick day rules
Antidiabetic medication • Already on one drug, but HbA1c has risen to 58mmol/mol (7.5%)
During a period of illness (that does not warrant admission), the patient should
(see Fig. 1 and Table 1 on Notes page overleaf) 53mmol/mol (7.0%) target
adhere to ‘sick day rules’ (see Ch5: Diabetes mellitus: type 1 management).
• HbA1c should be checked every 3–6 months until stable, then 6-monthly
• A more conservative approach should be taken for people who are older or
frail due to risk of hypoglycaemia Bariatric surgery
Self-monitoring • Bariatric surgery may be indicated for people with a BMI of 35 or over
• Bariatric surgery has been shown to induce remission of diabetes or reduce
Patients with T2DM do not need to routinely monitor their glucose unless: the need for medications with long-term results in morbidly obese patients
• The person is on insulin, or • The two common types of surgery are gastric banding and gastric bypass
• There is evidence of hypoglycaemic episodes, or surgery
• The person is on oral medication that may increase their risk of
hypoglycaemia while driving or operating machinery, or
• The person is pregnant or is planning to become pregnant
• Starting treatment with corticosteroids or to confirm suspected
hypoglycaemia
• There is acute intercurrent illness at risk of worsening hyperglycaemia;
review treatment as necessary

144 / Chapter 5 Endocrinology: Diabetes mellitus: type 2 management Mind Maps for Medicine \ 145
 Diabetes mellitus: type 2 management notes Table 1 T2DM medication

Drug Notes

T2DM pharmacological management algorithm Metformin • Methods of action ↓Hepatic gluconeogenesis, ↑tissue sensitivity to insulin, ↑peripheral glucose uptake
and use, ↓intestinal absorption of glucose
• Dosage Initially 500mg OD, maximum daily dose of 2g to be given in 2–3 divided doses
• Efficacy Moderate
Metformin 1st line • Advantages Weight reduction, cardiovascular (CV) benefit and hypoglycaemic risk is low
(unless contraindicated or not tolerated) • Disadvantages and common side effects GI side effects are common; a rare but important side effect
Check HbA1c after 3–6 months is lactic acidosis
• Cautions Avoid if eGFR is <30ml/min/1.73m2
Not reaching target
Sulfonylureas • Examples Gliclazide, glibenclamide, tolbutamide
• Dosage example Gliclazide initially 40–80mg OD, increased if necessary up to 160mg OD, maximum
320mg in 2–3 divided doses per day
Metformin + gliptin or
• Method of action Stimulate beta cells of the pancreas to release insulin
Metformin + sulfonylurea or
• Advantages High efficacy
Metformin + pioglitazone or
• Disadvantages and common side effects No CV benefit, side effects of weight gain and
Metformin + SGLT-2 inhibitor
hypoglycaemia
Check HbA1c after 3–6 months • Cautions Obesity, elderly, G6PD deficiency

Not reaching target Thiazolidinediones • Examples Pioglitazone is the only licensed medication from this class
• Dosage Initially 15–30mg OD, adjusted according to response
• Method of action Binds to and activates PPAR-gamma which is a nuclear receptor that regulates a large
number of genes including lipid metabolism and insulin action by reducing hepatic glucose production
Triple therapy and enhancing peripheral glucose uptake
Combination of any of the 3 above drugs • Advantages High efficacy
(including metformin) • Disadvantages and common side effects Causes weight gain, oedema and increased risk of fractures,
bladder cancer and heart failure
Check HbA1c after 3–6 months
SGLT-2 inhibitors • Examples Canagliflozin, dapagliflozin, empagliflozin
Not controlled with triple therapy • Dosage example Canagliflozin 100mg OD; increased if tolerated to 300mg OD if required
• Method of action Reduces renal tubular glucose reabsorption, producing a reduction in blood glucose
without stimulating insulin release
• Advantages CV benefit, causes weight loss and low risk of hypoglycaemia
Refer to diabetes specialist team, add another oral agent from • Disadvantages and common side effects Genital thrush is common. Can cause diabetic ketoacidosis.
a different class above or an injectable agent (dependent on BMI): • Cautions Avoid initiation if eGFR <60ml/min/1.73m2

DDP-4 inhibitors (gliptins) • Examples Sitagliptin, linagliptin

• Dosage example Sitagliptin 100mg OD
• Methods of action In normal physiology an oral glucose load results in a greater release of insulin than
if the same load is given intravenously – known as the incretin effect; this effect is largely mediated by
BMI >30kg/m2 BMI <30kg/m2 GLP-1 and is known to be decreased in T2DM; increasing GLP-1 levels either by the administration of an
analogue (GLP-1 mimetic, e.g. exenatide) or inhibiting its breakdown (DPP4 inhibitors – the gliptins) is
therefore the target of two recent classes of drug
Add GLP-1 agonist Add basal insulin • Advantages Weight neutral; well tolerated with few side effects
• Disadvantages and common side effects Low/moderate effect; no CV benefit
Fig. 1 Algorithm of the pharmacological management of T2DM; medication should be continued at each stage if either individualised HbA1c target reached or • Cautions Reduce dose in CKD stage 3
HbA1c falls >0.5% (5.5mmol/mol in 3–6 months); medication should be discontinued if ineffective or not tolerated; reinforce advice on diet, lifestyle and adherence to
antidiabetic drug treatment at each review; GLP-1, glucagon-like peptide-1; SGLT-2, sodium-glucose co-transporter 2
GLP-1 analogues • Examples Exenatide, dulaglitide, liraglutide
• Dosage example Exenatide initially 5µg BD for at least 1 month, then increased if necessary up to 10µg
BD by SC injection
• Method of action As above, GLP-1 stimulates the release of insulin
• Advantages High efficacy; has CV benefits; hypoglycaemic risk is low and causes weight loss; dose
unchanged in CKD
• Disadvantages and common side effects Main side effect is pancreatitis

146 / Chapter 5 Endocrinology: Diabetes mellitus: type 2 management notes Mind Maps for Medicine \ 147
 • Infection • Polyuria
DKA is a medical emergency characterised by the triad of • Glucose >11mmol/L or known • Cessation of insulin (unintentional or • Polydipsia
marked hyperglycaemia, acidosis and ketonaemia. diabetes mellitus deliberate) • Vomiting • Gastric stasis
It may be a complication of existing T1DM or be the • pH <7.3 or bicarbonate • Inadequate insulin • Dehydration • Thromboembolism
first presentation. DKA may also rarely occur in T2DM. It <15mmol/L • Cardiovascular disease e.g. stroke or • Altered mental state • Arrhythmias 2° to hyperkalaemia/
results from the absolute or relative deficiency of insulin • Ketones >3mmol/L or urine myocardial infarction • Coma iatrogenic hypokalaemia
in the presence of an increase in the counter-regulatory ketones ++ on dipstick • Drugs e.g. steroids, thiazides or • Weight loss • Iatrogenic cerebral oedema
hormones (glucagon, cortisol, growth hormone and sodium-glucose co-transporter 2 • Weakness • Hypokalaemia
adrenaline). Rarely, under conditions of extreme stress, (SGLT-2) inhibitors • Lethargy • Hypoglycaemia
patients with T2DM may also develop DKA. Diagnostic criteria Note: No obvious precipitant may be
• Kussmaul respiration • Acute respiratory distress syndrome
• Acetone breath smell • Acute kidney injury
present
Definition
Causes Clinical features Complications

Diabetic ketoacidosis

Investigations

Management 0.9% saline 1L over 1h

Bedside tests Venous blood gas

IV fluids 0.9% saline 1L with KCl over next 2h
Severity • Capillary blood glucose and ketone: • Shows metabolic acidosis with low pH
• Wide-bore cannula and bolus resuscitation significantly elevated and low HCO3– and hyperglycaemia
as required with 0.9% saline • Urine dipstick: ketone and glucose • Useful for working out anion gap:
0.9% saline 1L with KCl over next 2h ≥1 of the following may indicate positive, may show underlying infection • Anion gap = (Na+ + K+) – (Cl– + HCO3–)
• Followed by maintenance fluids (see Fig. 1)
• Maintain K+ between 4.0 and 5.5mmol/L severe DKA: • 12-lead ECG: rule out MI and arrhythmias • Usually >13 in DKA
(see Table 1) • Blood ketones >6mmol/L • Regular VBGs vital for monitoring
• HCO3– level <5mmol/L Blood tests
0.9% saline 1L with KCl over next 4h
Insulin • Venous/arterial pH <7.0 Imaging
• Hypokalaemia on admission • Plasma glucose Elevated
Fixed rate IV insulin infusion (FRIII) should be • Glasgow Coma Scale (GCS) score <12 • FBC ↑WCC in sepsis • CXR Look for source of infection
administered: 50 units human soluble insulin 0.9% saline 1L with KCl over next 4h • O2 saturation <92% • CRP ↑ In sepsis • AXR If indicated by history/examination
(Actrapid, Humulin S) made up to 50ml with • Systolic BP <90mmHg • U&Es Na+ may be high (dehydration) • CT/MRI If there is impairment of
0.9% NaCl; infuse at a fixed rate of 0.1U/kg/h. If • HR >100 or <60 or low due to interference of glucose/ consciousness or focal neurology
the patient normally takes long-acting insulin SC, • Anion gap >16 ketones, or normal; K+ may be ↑ due to
continue this at the usual dose and usual time. 0.9% saline 1L with KCl over next 6h acidosis, normal or occasionally low; urea
and creatinine may be raised
Monitoring Fig. 1 Example of IV fluid maintenance regime in DKA • Plasma osmolality >290mOsm/kg in
cases of DKA; if new presentation and
• Measure blood ketones and capillary glucose osmolality >320mOsm/kg and there is
hourly Table1 Potassium replacement in DKA not significant ketonaemia/ketonuria then
• Check the rates of ketone fall, glucose fall and hyperosmolar hyperglycaemic state (HHS)
bicarbonate rise K+ level in first 24h (mmol/L) K+ replacement in infusion solution
may be the diagnosis
• Measure venous blood gas (VBG) for pH, HCO3– • Cardiac enzymes (e.g. troponin) If
and K+ at 60 min, 2h and 2h thereafter >5.5 Nil
MI suspected, creatine kinase is raised if
• Continue the FRIII until the ketone rhabdomyolysis co-exists
measurement is <0.6mmol/L, venous pH >7.3 3.5–5.5 40mmol/L • Amylase If pancreatitis suspected
and/or venous HCO3– >18mmol/L • Blood cultures If sepsis suspected
• If the glucose falls <14.0mmol/L, commence <3.5 Senior review as additional potassium
10% glucose given at 125ml/h alongside the required (via central line in HDU)
0.9% NaCl

148 / Chapter 5 Endocrinology: Diabetic ketoacidosis Mind Maps for Medicine \ 149
 Hyperosmolar hyperglycaemic state Notes
Hyperosmolar hyperglycaemic state

Definition Investigations

Hyperosmolar hyperglycaemic state (HHS), formerly known as Same as for DKA

hyperosmolar hyperglycaemic non-ketotic coma (HONK) is a potentially
life-threatening illness that occurs in T2DM. It is characterised by severe Management
hyperglycaemia, hypovolaemia and raised plasma osmolality. Ketosis does
not occur due to the presence of basal insulin secretion sufficient to prevent • Fluid replacement IV 0.9% NaCl with K+ added as required as with DKA;
ketogenesis. A mixed picture of HHS and DKA may however occur. fluid replacement will reduce blood glucose; a rapid fall should be avoided
(aim for a reduction of 4–6mmol/h)
Precipitating factors • Insulin Fixed rate intravenous insulin infusion (FRIII) (0.05U/kg/h) is
recommended, ideally reducing blood glucose by up to 5mmol/L/h;
Intercurrent or co-existing illness reassess fluid intake and renal function once blood glucose has ceased to
• MI decline during initial fluid replacement
• Infection (most common): e.g. UTI, pneumonia, cellulitis, systemic sepsis • Treatment in HDU in severe cases (see below)
• Stroke/TIA/intracranial haemorrhage • Regular monitoring VBGs as with DKA
• Hyperthermia or hypothermia • Antibiotics Give antibiotics when there is clinical, imaging or laboratory
• Endocrine: hyperthyroidism, Cushing syndrome or ACTH-secreting tumour evidence of infection
• Intestinal ischaemia/infarction • Anticoagulation Patients in HHS are at increased thrombotic risk and
• AKI or decompensated CKD should receive prophylactic treatment for this (LMWH) throughout
• Pancreatitis admission, unless contraindicated
• PE
• Burns Care in HDU/ITU should be considered if ≥1 of following:
• Osmolality >350mOsm/kg
Drugs • Na+ >160mmol/L
• Calcium channel blockers • Venous/arterial pH <7.1
• Beta blockers • Hypokalaemia (<3.5mmol/L) or hyperkalaemia (>6mmol/L) on admission
• Chemotherapeutic agents • GCS <12 or abnormal AVPU (alert, voice, pain, unresponsive) scale
• Glucocorticoids • Oxygen saturation <92% on air (assuming normal baseline respiratory
• Loop diuretics function)
• Alcohol and illicit drugs including cocaine, amphetamines and MDMA • SBP <90mmHg
(Ecstasy) • Pulse >100 or <60bpm
• Urine output <0.5ml/kg/h
• Serum creatinine >200µmol/L
Diabetes related • Hypothermia
• First presentation of diabetes mellitus: unsuspected, undiagnosed • Macrovascular event (e.g. myocardial infarction or stroke)
• Poor diabetic control/non-compliance: intentional, accidental, neglect • Other serious comorbidity

Clinical features Complications

The patient is often elderly and may be presenting for the first time: • Ischaemia or infarction affecting any organ, particularly MI and
• Hyperglycaemia cerebrovascular event
• Dehydration with marked thirst • Thromboembolic disease, including DVT and PE
• Marked drowsiness • Renal failure and multi-organ failure
• Convulsions, coma and focal CNS signs • Acute respiratory distress syndrome
• Disseminated intravascular coagulation
Diagnostic features • Rhabdomyolysis
• Cerebral oedema
• Hypovolaemia
• Central pontine myelinolysis
• Marked hyperglycaemia (≥30mmol/L) without significant
• Iatrogenic complications: hypoglycaemia due to over-administration
hyperketonaemia (7.3, HCO3– >15mmol/L)
of insulin; fluid overload leading to cardiac failure
• Raised plasma osmolality usually ≥320mOsm/kg

150 / Chapter 5 Endocrinology: Hyperosmolar hyperglycaemic state Mind Maps for Medicine \ 151
 • Aldosterone is a steroid hormone and is the main Primary hyperaldosteronism Secondary hyperaldosteronism
mineralocorticoid secreted by the zona glomerulosa of
the adrenal cortex • Adrenal adenoma (Conn syndrome) Accounts for >80% of all cases of Caused by ↑renin due to ↓renal perfusion:
• The control of aldosterone synthesis and release is through the hyperaldosteronism; usually unilateral and solitary • Renal artery stenosis
renin–angiotensin–aldosterone system (RAAS) • Adrenal hyperplasia Accounts for approx. 15% of all cases of • Diuretics
• The most important physiological effect of aldosterone is hyperaldosteronism; majority of cases are bilateral • CCF
stimulation of Na+ reabsorption and K+ excretion in the late distal • Familial hyperaldosteronism There are 2 forms: type 1 is glucocorticoid- • Hepatic failure
Hyperaldosteronism can be defined tubule and collecting duct, thereby indirectly influencing water remediable aldosteronism (GRA) and type 2 is characterised by inherited • Nephrotic syndrome
as excessive levels of aldosterone retention or loss, blood pressure and blood volume aldosterone-producing adenoma or inherited bilateral adrenal hyperplasia • Malignant hypertension
which may be independent of the • Hyperaldosteronism therefore causes high plasma Na+, • Adrenal carcinoma A rare cause of primary hyperaldosteronism
renin–angiotensin axis (primary hypertension and low K+
hyperaldosteronism) or due to • This may be independent of the RAAS (primary
elevated renin levels (secondary hyperaldosteronism) or due to elevated renin levels (secondary
hyperaldosteronism)
Causes
hyperaldosteronism).

Definition Physiology Notes

Hyperaldosteronism

Hyperaldosteronism

Management Investigations Clinical features

• Conn syndrome Laparoscopic • U&Es May show hypokalaemia • Hypertension Usually

adrenalectomy; medical and hypernatraemia asymptomatic
management with spironolactone • Plasma aldosterone : renin • Hypokalaemia Weakness,
is used in the period prior to ratio Raised cramps, paraesthesia, polyuria,
surgery • Blood gas Hypokalaemia, polydipsia
• Hyperplasia Managed with alkalosis • Metabolic alkalosis
spironolactone, amiloride or • ECG May show arrhythmias from • Sodium may be raised
eplerenone electrolyte imbalance
• GRA Dexamethasone for 4 weeks • CT/MRI adrenal glands To locate
• Adrenal carcinoma Surgically an adrenal adenoma, hyperplasia
treated or carcinoma
• Selective adrenal villous
sampling Gold standard for
localising the cause of primary
hyperaldosteronism
• Genetic testing Available for GRA

152 / Chapter 5 Endocrinology: Hyperaldosteronism Mind Maps for Medicine \ 153

 Sunlight
↓PTH Other causes
• Serum calcium levels are
regulated by PTH and vitamin D Skin • Parathyroid destruction: surgery (thyroidectomy, parathyroidectomy, • Hyperventilation
on the kidneys, bone and GI tract 7-dehydrocholesterol radical neck dissection), radiotherapy, infiltration by metastases or • Drugs: calcium chelators, bone
(see Fig. 1) systemic disease (e.g. sarcoidosis, amyloidosis, HIV, syphilis) resorption inhibitors (bisphosphonates,
• PTH increases calcium levels by • Parathyroid agenesis calcitonin), phenytoin, ketoconazole,
increasing reabsorption of calcium Food Vitamin D • Reduced parathyroid secretion: due to gene defects e.g. foscarnet
and activation of vitamin D in the DiGeorge syndrome, hypomagnesaemia, hungry bone disease (after • Acute pancreatitis
kidneys and stimulating calcium Liver parathyroidectomy), mutation in calcium-sensing receptor (CASR) • Acute rhabdomyolysis
release from bone (25-hydroxylase) • Isolated autoimmune hypoparathyroidism • Malignancy: tumour lysis syndrome,
• The active form of vitamin D osteoblastic metastases
Hypocalcaemia is a low plasma (1,25-dihydroxyvitamin D) ↑PTH • Toxic shock syndrome
calcium i.e. <2.2mmol/L. increases intestinal calcium 25(OH)D
Hypocalcaemia is nearly always due absorption • Vitamin D deficiency: due to nutritional lack, malabsorption, liver
to deficiency or resistance to PTH or • The normal range of calcium is Kidney disease, receptor defects
vitamin D deficiency. 2.2–2.6mmol/L PTH activates • Vitamin D resistance: renal tubular dysfunction (Fanconi syndrome) or
receptor defect
• PTH resistance: pseudohypoparathyroidism, hypomagnesaemia
(1-hydroxylase)
Causes
Definition Calcium physiology

1,25(OH)2D

Fig. 1 Calcium homeostasis

Hypocalcaemia
Notes
Hypocalcaemia

Management Investigations Clinical features

Acute treatment • Calcium (adjusted) <2.2mmol/L Symptoms Signs

• Phosphate ↑ In CKD and hypoparathyroidism and
• Treat where symptomatic or severe ↓ in vitamin D deficiency • Usually asymptomatic • Chvostek’s sign (see Fig. 2a)
hypocalcaemia i.e. <1.90mmol/L • ALP ↑ In CKD and vitamin D deficiency • Paraesthesia (usually fingers, toes • Trousseau’s sign (see Fig. 2b)
• Treat with calcium bolus i.e. 10ml of 10% • Vitamin D To exclude vitamin D deficiency and around mouth) • Seizures
calcium gluconate over 10min (repeat as • U&Es To exclude CKD • Tetany • Ventricular fibrillation (VF) or heart
necessary) or calcium infusion • PTH To evaluate likely cause • Carpopedal spasm (wrist flexion block
• Oral calcium may be needed in addition • CK To exclude rhabdomyolysis and fingers drawn together) • Laryngospasm/bronchospasm
• Monitor serum calcium concentrations regularly • Amylase To exclude pancreatitis • Muscle cramps • With prolonged hypocalcaemia:
(ITU monitoring may be required) • ECG To exclude dysrhythmias and prolonged subcapsular infarct, papilloedema,
• Correct magnesium if low QT interval abnormal teeth, confusion
• X-ray hand Shorter 4th and 5th metacarpals in
Long-term treatment/prevention pseudohypoparathyroidism
• ECHO Cardiac structural defects in DiGeorge syndrome Fig. 2 (a) Chvostek’s sign (muscular
• Oral calcium replacement e.g. with calcium spasm provoked by tapping of the
carbonate facial nerve below the zygomatic arch);
• Oral vitamin D replacement: cholecalciferol (b) Trousseau’s sign (carpopedal spasm
• Ensure adequate dietary intake: foods rich in provoked by inflating a blood pressure
calcium or supplements (a) (b) cuff for several minutes)
• Monitor those at risk

154 / Chapter 5 Endocrinology: Hypocalcaemia Mind Maps for Medicine \ 155

 PTH mediated Primary hyperparathyroidism

• Primary hyperparathyroidism Most common cause of hypercalcaemia • Most common cause of hypercalcaemia
• Familial MEN-1 and MEN-2A, familial hypocalciuric hypercalcaemia • Causes: adenoma (80–85%), multiple gland hyperplasia
• Tertiary hyperparathyroidism Secondary to acute renal failure (10–15%), parathyroid cancer (<1%)
• Diagnosis: ↑serum Ca2+ and ↑PTH; ultrasound or technetium
• Serum calcium levels are regulated by PTH and Non PTH mediated scan of the parathyroid glands
Hypercalcaemia is elevated calcium levels, commonly vitamin D on the kidneys, bone and gastrointestinal
(GI) tract • Malignancy Common; mechanism is through parathyroid-related Secondary hyperparathyroidism
caused by an excessive release of PTH or it may be
• PTH causes increased calcium levels by increasing peptide or bone metastases
independent of PTH.
reabsorption of calcium and activation of vitamin D in • Medications Thiazide diuretics, lithium, teriparatide, excess vitamin D, • Not a cause of hypercalcaemia but an appropriate increase in
• Mild hypercalcaemia 2.65–3.00mmol/L
the kidneys and increased calcium release from bone; excess vitamin A, theophylline toxicity PTH in response to low Ca2+
• Moderate hypercalcaemia 3.01–3.40mmol/L
the active form of vitamin D (1,25-dihydroxyvitamin D, • Chronic granulomatous conditions Sarcoidosis, TB • Causes: most commonly chronic renal failure or vitamin D
• Severe hypercalcaemia >3.40mmol/L
calcitriol) promotes intestinal calcium absorption • Endocrine conditions Hyperthyroidism, acromegaly, deficiency
• Normal calcium range is 2.2–2.6mmol/L phaeochromocytoma, adrenal insufficiency • Calcium will be low or normal in presence of raised PTH;
• Miscellaneous Immobilisation, milk alkali syndromes vitamin D is usually low and phosphate high
Definition Normal physiology Tertiary hyperparathyroidism
Causes • Progression of secondary hyperparathyroidism to
an autonomous overproduction of PTH resulting in
hypercalcaemia
• Differs from primary hyperparathyroidism as phosphate
often remains elevated

Hypercalcaemia and hyperparathyroidism Hyperparathyroidism

Notes
Hypercalcaemia and hyperparathyroidism

Management Investigations Clinical features

• Stop offending drugs toxicity, sarcoidosis and • PTH Raised in primary, secondary and tertiary ‘Bones, stones, abdominal groans and
• IV fluids 0.9% saline helps to lymphoma hyperparathyroidism and decreased in vitamin D excess/ psychic moans’
increase the urinary output of • Cinacalcet hydrochloride milk alkali syndrome, granulomatous disease, adrenal • GI Anorexia, nausea, vomiting,
calcium and should be used 1st A calcimimetic agent that insufficiency and thyrotoxicosis constipation, pancreatitis, peptic ulcer
line in patients with moderate effectively reduces PTH levels • Serum phosphate Usually low in primary disease
and severe hypercalcaemia in patients with secondary hyperparathyroidism and raised in secondary and tertiary • Musculoskeletal Muscle weakness,
• Bisphosphonates After hyperparathyroidism hyperparathyroidism bone pain, osteopenia/osteoporosis
rehydration, bisphosphonates • Paricalcitol Licensed for the • Serum ALP Raised in malignancy (not haematological • Renal Polyuria, polydipsia, kidney
may be administered; IV prevention and treatment of malignancy) stones, distal renal tubular acidosis,
pamidronate and zoledronic acid secondary hyperparathyroidism • Vitamin D Raised in vitamin D intoxication nephrogenic diabetes insipidus
are commonly used associated with CKD • Urine calcium Low in familial hypocalciuric hypercalcaemia • Neurological Decreased
• Loop diuretics e.g. Furosemide • Denosumab Useful for patients • CXR To rule out lung malignancy, sarcoidosis, TB concentration, confusion, fatigue,
is occasionally used with persistent or relapsed • U&Es To assess renal function and dehydration stupor, coma
• Treat underlying cause hypercalcaemia of malignancy • FBC May show iron deficiency anaemia or anaemia of • Cardiovascular Cardiac arrhythmias,
e.g. Malignancy • Haemodialysis Should be chronic disease shortened QT interval
• Calcitonin Has fewer side effects considered for patients with • Serum electrophoresis To rule out myeloma
than bisphosphonates but is less advanced underlying kidney • Plain X-ray May show features indicative of bone
effective in reducing calcium disease and refractory severe abnormalities e.g. demineralisation, bone cysts, pathological
levels hypercalcaemia fractures or bony metastases
• Glucocorticoids Useful for • ECG Severe hypercalcaemia can cause cardiac arrhythmias
hypercalcaemia due to vitamin D and shortened QT interval
• US technetium scan Of parathyroid glands if
hyperparathyroidism is suspected

156 / Chapter 5 Endocrinology: Hypercalcaemia and hyperparathyroidism Mind Maps for Medicine \ 157
 • Prolactin is primarily
synthesised and released Suckling Diurnal rhythm • Unlike other hormones of the anterior pituitary gland, • Physiological causes Stress (physical or psychological), pregnancy,
by the lactotrophs of the + + prolactin secretion is mainly under inhibitory control breastfeeding
anterior pituitary gland (predominantly by dopamine) rather than secretion from a • Hypothalamus disease Trauma, radiotherapy, infiltration (sarcoidosis,
although extrapituitary hypothalamic-releasing hormone histiocytosis), tumours e.g. craniopharyngioma, metastatic disease
prolactin synthesis is also Hypothalamus • Hyperprolactinaemia can be caused by a number of stimuli • Pituitary disease Prolactinomas (micro- or macro-), mixed lactotroph/
recognised in immune, – of prolactin release e.g. prolactinomas or loss of dopamine mammotroph adenoma, stalk compression (from any pituitary/sellar
mammary, epithelial and TRH + – Dopamine inhibition pathology)
Hyperprolactinaemia is defined fat cells • The loss of dopamine inhibition may be due to a true • Endocrine Hypothyroidism (due to ↑ synthesis of thyrotropin-releasing
as a raised level of prolactin in the • The control of prolactin – dopamine deficiency (e.g. tumours of the hypothalamus), hormone, TRH), Cushing syndrome, PCOS, acromegaly
Pituitary gland (lactotroph)
blood. Hyperprolactinaemia can be secretion is highlighted a defect in transport of dopamine from hypothalamus to the • Drugs Oestrogens, anti-emetics (domperidone, metoclopramide),
physiological, e.g. during pregnancy in Fig. 1 pituitary gland (e.g. a pituitary or stalk tumour), antagonism antipsychotics e.g. haloperidol, antidepressants (SSRIs), opioids
and stress, or pathological. • In humans, the only clearly of dopamine by certain drugs (e.g. antipsychotics) • Impairment of metabolism/excretion Chronic renal failure or severe liver
defined physiological role Prolactin or unregulated stimulation of lactotrophs e.g. chest wall disease
of prolactin is stimulation of injury resulting in mimicking of ‘suckling’ reflex • Chest wall trauma or surgery
lactation during pregnancy
Mammary gland development
Definition when prolactin levels
and lactation
are raised
Pathophysiology Causes
Fig. 1 Control of prolactin secretion

Normal physiology

Hyperprolactinaemia

Complications
Management Investigations Clinical features
Related to hypogonadism
Conservative • TFTs To exclude hypothyroidism as a cause
• Osteoporosis Related to tumour size (usually macroadenomas)
of ↑prolactin
• ↓Fertility • Beta HCG (urine or serum) To exclude
• Observation • Headache
• Erectile dysfunction pregnancy
• Microadenomas in asymptomatic patients may not need observation • Visual disturbances (typically bitemporal hemianopia or upper temporal
• Stop offending drugs (if possible) • Basal serum prolactin (non-stressful
Related to tumour size quadrantanopia)
• Treat underlying reversible causes e.g. hypothyroidism venepuncture) If mildly elevated, it should • Cranial nerve palsies
• Visual loss be repeated, a prolactin level >5000mU/L • Symptoms/signs of hypopituitarism
• Headache Medical usually indicates a true prolactinoma
• Pituitary apoplexy • Visual field assessment Often bitemporal Related to hypogonadism
• Dopamine agonists e.g. cabergoline, bromocriptine hemianopia
• CSF rhinorrhoea
• Exclude cardiac valve fibrosis and pulmonary fibrosis before starting • MRI pituitary To identify a pituitary tumour Males Females
treatment
• Reduced libido • Amenorrhoea/oligomenorrhoea
• After 1 month of therapy, the patient should be evaluated for side
• Reduced beard growth • Galactorrhoea
effects and serum prolactin should be measured
• Erectile dysfunction • Subfertility
Surgical • Galactorrhoea • Hirsutism
• Reduced libido
• Recommended on basis of tumour size and symptoms, inadequate
response or intolerant to dopamine agonists
• Often using a trans-sphenoidal route to remove a tumour
• Combined with postoperative radiotherapy for large tumours, often
restores normoprolactinaemia
• Unfortunately there is a high risk of recurrence

158 / Chapter 5 Endocrinology: Hyperprolactinaemia Mind Maps for Medicine \ 159

 • The thyroid gland is a bi-lobed structure which is found in the • Iodine deficiency is the most common preformed T4 and T3 may ‘leak’ into the Primary
anterior neck (see Fig. 1) Stress Cold cause of primary hypothyroidism worldwide circulation from damaged cells causing
• The hypothalamus secretes thyrotropin-releasing hormone • Hashimoto’s thyroiditis is the most transient hyperthyroidism • Hashimoto’s thyroiditis
(TRH) which stimulates the anterior pituitary to secrete thyroid- + + common cause of primary goitrous • It is associated with HLA-DR5; • De Quervain’s thyroiditis: associated
stimulating hormone (TSH); this then acts on the thyroid gland hypothyroidism in non-iodine deficient areas: monozygotic twin studies indicate that with a painful goitre and raised ESR
increasing the production of thyroxine (T4) and tri-iodothyronine Hypothalamus – • There is autoimmune destruction of there is about 70% genetic contribution • Riedel thyroiditis: fibrous tissue
(T3), the two main thyroid hormones (see Fig. 2) thyroid cells by cell- and antibody- to autoimmune thyroid disease replacing the normal thyroid
• These then act on a wide variety of tissues, helping to regulate mediated immune processes • It is 5–10 × more common in women parenchyma; it causes a painless goitre
the use of energy sources, protein synthesis, and control the TRH • The major environmental triggers of than men • Post-partum thyroiditis
body’s sensitivity to other hormones autoimmune thyroid disease include • It is associated with other autoimmune • Drugs: amiodarone, contrast media,
iodine, medications, infection, smoking disorders e.g. IDDM, Addison’s and iodides, lithium and antithyroid
Epiglottis –
Hypothyroidism is the clinical result Anterior pituitary gland and possibly stress pernicious anaemia medication
Hyoid bone
of impaired production of the • Most patients have serum antibodies • Secondary hypothyroidism is rare and • Iodine deficiency
Thyroid cartilage to thyroglobulin, thyroid peroxidase produces disruption in the feedback loop
thyroid hormones, thyroxine (T4) • Infiltration of thyroid: e.g.
Pyramidal lobe TSH enzyme and antibodies that block TSH because of problems at the level of the
and tri-iodothyronine (T3), which amyloidosis, sarcoidosis and
of thyroid gland to its receptor pituitary gland
are essential for normal growth, haemochromatosis
development and metabolism. LEFT LOBE OF • The result is inadequate thyroid hormone • Post thyroidectomy/radioactive
RIGHT LOBE OF Thyroid gland
THYROID GLAND secretion, although, initially both iodine therapy
THYROID GLAND
Isthmus of thyroid • Congenital hypothyroidism: due to
Definition gland
Pathophysiology a problem with thyroid dysgenesis or
thyroid dyshormonogenesis
Trachea
T4, T3 Secondary

Fig. 1 Location of the thyroid gland and neighbouring structures Fig. 2 The hypothalamus–pituitary–thyroid axis • Isolated TSH deficiency
• Hypopituitarism: neoplasm,
infiltrative, infection e.g. TB, Sheehan
Thyroid physiology syndrome, radiotherapy
• Hypothalamic disorders: neoplasms
and trauma
• Impaired quality of life
• Cardiovascular complications:
dyslipidaemia, coronary artery Causes
disease, heart failure
• Hyperthyroidism (from
overtreatment of hypothyroidism)
Hypothyroidism
• Reproductive complications:
impaired fertility, obstetric
complications e.g. pre-eclampsia
• Myxoedema coma

Complications
Clinical features

Symptoms Signs (see Fig. 3)

Management Investigations Hair dry,
coarse, sparse
• Tiredness, lethargy • Dry coarse skin
• Intolerance to cold • Hair loss (typically scalp and lateral third
Thyroxine replacement • Thyroid function tests (TFTs): • Dry skin and hair loss of eyebrow) Lateral
• 1° hypothyroidism: ↑TSH, ↓T3, T4 • Slowing of intellectual activity • Cold peripheries eyebrows thin
• Lifelong replacement with levothyroxine is the mainstay of treatment • 2° hypothyroidism: ↓TSH, ↓T3, T4 • Constipation • Puffy face, hands and feet (myxoedema) Periorbital
• For adults aged over 18 years, the initial dose of levothyroxine is • Subclinical hypothyroidism: ↑TSH, →T3, T4 • Decreased appetite with weight gain • Bradycardia oedema
50–100µg once daily • Autoantibodies Antithyroid peroxidase (anti-TPO) antibodies or • Deep hoarse voice • Delayed tendon reflex relaxation
• There are certain patients for whom the recommended initial dose of antithyroglobulin antibodies are found in 90–95% of patients with • Menorrhagia and later oligomenorrhoea or • Carpal tunnel syndrome
Puffy dull face
levothyroxine is 25µg, including: autoimmune thyroiditis; TSH receptor antibodies may also be found amenorrhoea • Serous cavity effusions: e.g. pericarditis
with dry skin
• Patients with cardiac disease • FBC, lipid profile, CK Untreated hypothyroidism can cause ↑CK, • Impaired hearing due to fluid in middle ear or pleural effusions
• Patients with severe hypothyroidism ↑cholesterol and triglycerides and anaemia (normocytic or macrocytic) • Reduced libido
• Patients aged over 50 years • Imaging E.g. ultrasonography, to rule out neoplastic lesions if the patient
• Once TFTs are stabilised, TSH should be checked annually has an asymmetrical goitre Fig. 3 Facial signs of hypothyroidism

160 / Chapter 5 Endocrinology: Hypothyroidism Mind Maps for Medicine \ 161

 Hypothyroidism notes Notes
Hypothyroidism

Interpreting thyroid function tests Subclinical hypothyroidism

• Subclinical hypothyroidism occurs when a patient has a TSH level above the
Diagnosis TSH Free T4 upper limit of the reference range and free T4 levels are within the normal
reference range
Thyrotoxicosis ↓ ↑ • There is some controversy over whether to treat these patients; symptoms
may improve on T4 but there are concerns over risk of reduced bone mineral
Primary hypothyroidism ↑ ↓ density and atrial fibrillation
• The following patients should be considered for treatment with
Secondary hypothyroidism ↓ ↓ levothyroxine:
• Those with TSH level >10mU/L
Sick euthyroid syndrome* ↓ ↓ • Those who are symptomatic
• Those with a history of radio-iodine treatment or positive thyroid
↑ → antibody test (as this subgroup almost always progress to overt
Subclinical hypothyroidism**
hypothyroidism)
• If there has been previous treatment of Graves’ disease or other organ-
Poor compliance with thyroxine*** ↑ →
specific autoimmune disease
*Common in hospital inpatients; changes are reversible upon recovery from • If none of the above is present, then TSH should be monitored every
the systemic illness and no treatment is usually needed 6–12 months
**Common finding and represents patients who are likely to go on and
develop hypothyroidism but still have normal thyroxine levels Hypothyroidism in pregnancy
***Patients who are poorly compliant may only take their thyroxine in the days • Women of childbearing age should be encouraged to wait until they are
before a routine blood test; the thyroxine levels are hence normal but the TSH euthyroid prior to trying to conceive
‘lags’ and reflects longer-term low thyroxine levels • It is important to maintain a euthyroid state throughout pregnancy,
especially during the first trimester
Myxoedema coma • TFTs should be measured during the 1st, 2nd and 3rd trimesters for all
• Myxoedema coma is a medical emergency seen mostly in elderly patients pregnant women with hypothyroidism (TSH is a sensitive marker of thyroid
and is associated with a high mortality rate dysfunction during pregnancy)
• Patients may be on treatment for hypothyroidism or be previously • Treating clinical and subclinical hypothyroidism can reduce adverse obstetric
undiagnosed outcomes
• Infections and discontinuation of thyroid supplements are the major • Levothyroxine dose may need to be increased by more than 50% during
precipitating factors pregnancy; the dose can usually be reduced postpartum

Clinical features

• A reduced level of consciousness

• Seizures
• Hypothermia
• Features of hypothyroidism

Management

• IV levothyroxine is the mainstay of therapy

• T3 therapy may also be used but this can cause arrhythmias
• Supportive therapy, e.g. IV fluids, correction of metabolic disturbances,
hypothermia correction
• Intubation and ventilation if respiratory impairment is severe
• IV hydrocortisone as impaired adrenal function is present in profound
hypothyroidism

162 / Chapter 5 Endocrinology: Hypothyroidism notes Mind Maps for Medicine \ 163
 • Graves’ disease: the hyperthyroidism that probably
most common cause of results from a viral infection; often
• Primary hyperthyroidism is the term used when the
thyrotoxicosis; as well accompanied by pyrexia and a
pathology is within the thyroid gland
as typical features of painful goitre (see Fig. 1)
• Secondary hyperthyroidism is rare and occurs when the
thyrotoxicosis other features • Other forms of thyroiditis:
thyroid gland is stimulated by excessive thyroid-stimulating
may be seen including subacute thyroiditis, silent
hormone (TSH) in the circulation due to pathology of the
thyroid eye disease thyroiditis and postpartum
pituitary gland
• Toxic multinodular goitre: thyroiditis
• Graves’ disease is the most common cause of primary
autonomously functioning • Drugs: e.g. amiodarone, lithium,
hyperthyroidism and is the result of IgG antibodies
Hyperthyroidism occurs when thyroid nodules that secrete exogenous iodine and thyroid
binding to the TSH receptor and stimulating excess thyroid
an excess of circulating thyroid excess thyroid hormones hormone Fig. 1 Thyroid goitre
hormone production
hormones (thyrotoxicosis) is • Solitary thyroid nodule: • Follicular carcinoma of the
• TSH receptor stimulating antibodies and antithyroid
produced by an overactive thyroid palpable, toxic adenoma thyroid gland: associated with
peroxidase antibodies are present in most patients with Graves’
• De Quervain’s thyroiditis: metastatic disease
gland. disease
a transient form of • TSH-secreting pituitary Causes
• Graves’ disease is associated with other autoimmune disorders
adenoma
e.g. pernicious anaemia and myasthenia gravis
Definition

Pathophysiology

• Atrial fibrillation
• High-output cardiac failure
• Osteoporosis
• Cardiomyopathy
• Thyroid storm Hyperthyroidism Clinical features
Complications
Symptoms Signs

• Weight loss or gain • Palmar erythema

Investigations • Increased or decreased appetite • Sweaty and warm palms
• Irritability • Fine tremor
Conservative Management •
•
Weakness and fatigue
Diarrhoea ± steatorrhoea
• Tachycardia: may present as atrial fibrillation and/or
heart failure (common in the elderly)
• Thyroid function tests (TFTs)
• Education • Sweating • Hair thinning or diffuse alopecia
(see Notes)
• Smoking cessation • Tremor • Pretibial myxoedema (see Fig. 2)
• Autoantibodies Antimicrosomal
• Mental illness: anxiety, • Thyroid acropachy
Pharmacological Radioiodine antibodies against thyroid
depression, psychosis • Urticaria, pruritus
peroxidase, antithyroglobulin
• Heat intolerance • Brisk reflexes
• Symptomatic control Propranolol for tremor and palpitations • Usually the treatment of choice in relapsed Graves’ disease antibodies and TSH-receptor
• Loss of libido • Goitre
• Antithyroid drugs (carbimazole or propylthiouracil): and in those patients with toxic nodular hyperthyroidism antibodies are all positive in most
• Oligomenorrhoea or • Anxiety
• Act very quickly and inhibit the production of thyroid hormones • Radioactive iodine is given to the patient as a drink and is patients with Graves’ disease
amenorrhoea • Proximal myopathy (muscle weakness ± wasting)
• Two potential methods of treating hyperthyroid patients: ‘block and taken up by the thyroid gland, causing destruction of the • Imaging:
• Gynaecomastia
replace’ (antithyroid drugs are given with thyroxine replacement), and gland; it can take up to 3–4 months to take effect • US scan of nodules
• Lid lag (may be present in any cause of hyperthyroidism)
‘dose titration’ (only antithyroid drugs are used and doses are adjusted to • It is contraindicated in pregnancy and breastfeeding, and • Isotope scan to locate hot
• Thyroid eye disease (see Notes) including exophthalmos
achieve normalisation of TFTs); both methods are equally effective women must be advised not to get pregnant for at least (overactivity) and cold (no
(see Fig. 3) and ophthalmoplegia
• Carbimazole is usually used 1st line; agranulocytosis is an important 6 months activity) spots
adverse effect and patients should be warned to seek urgent medical • Hypothyroidism is a potential and common complication • Inflammatory markers CRP and
help if they develop a fever, sore throat etc; it is teratogenic and therefore ESR are often raised in patients
women of childbearing potential should use effective contraception Surgical with subacute thyroiditis
during treatment • Fine-needle aspiration Aspirate
• Propylthiouracil Known to cause severe liver failure, particularly in • Subtotal or near total thyroidectomy achieves a 98% nodules to rule out malignancy
children, and therefore reserved for use in pregnancy and thyroid storm cure rate; it is indicated if there is suboptimal response
to antithyroid medication or radioiodine (particularly in
patients who are pregnant or who have Graves’ orbitopathy)
• Patients should be returned to the euthyroid state with
antithyroid drugs before surgery to avoid thyroid storm
• Complications are rare but include haemorrhage,
hypoparathyroidism and vocal cord paralysis
Fig. 2 Pretibial myxoedema Fig. 3 Exophthalmos

164 / Chapter 5 Endocrinology: Hyperthyroidism Mind Maps for Medicine \ 165

 Hyperthyroidism notes Notes
Hyperthyroidism

Thyroid eye disease Thyroid storm

Thyroid eye disease affects between 25 and 50% of patients with Graves’ Hyperthyroid crisis, or thyrotoxic storm, is an extreme manifestation of
disease and patients may be eu-, hypo- or hyperthyroid at the time of thyrotoxicosis due to overproduction of thyroid hormones; it classically occurs
presentation. in patients with underlying Graves’ disease or toxic multinodular goitre.

Pathophysiology Presentation

• It is thought to be caused by an autoimmune response against an Often, there is sudden onset of severe hyperthyroidism with:
autoantigen, possibly the TSH receptor causing retro-orbital inflammation • Hyperpyrexia (>41°C), dehydration
• Smoking is the most important modifiable risk factor for the development of • Heart rate >140bpm (with or without AF/other arrhythmias), hypotension,
thyroid eye disease congestive heart failure
• Radioiodine treatment may increase the inflammatory symptoms seen in • Nausea, jaundice, vomiting, diarrhoea, abdominal pain
thyroid eye disease • Confusion, agitation, delirium, psychosis, seizures or coma

Clinical features Common precipitants

• Exophthalmos • Infection or other acute illness

• Conjunctival oedema • Withdrawal of or non-compliance with antithyroid medication
• Optic disc swelling • Recent trauma, surgical stress, recent thyroid surgery
• Ophthalmoplegia • MI, stroke, pulmonary embolism
• Increased risk of exposure keratopathy • Diabetic ketoacidosis, hyperosmolar coma or hypoglycaemia
• Following childbirth
Management • Drugs: radioiodine, amiodarone, radiographic contrast media
• Overdose of thyroid hormone tablets
• Smoking cessation • Vigorous palpation of the thyroid gland in hyperthyroid patients
• Topical lubricants to help prevent corneal inflammation caused by exposure
• High-dose systemic steroids e.g. prednisolone to reduce inflammation Management
• Radiotherapy
• Surgery • Treatment of the precipitating cause, e.g. any suspected infection
• Resuscitation: oxygen, IV fluids and nasogastric tube if there is vomiting
• Antithyroid treatment: carbimazole or propylthiouracil orally
Subclinical hyperthyroidism • Lugol’s solution (aqueous iodine oral solution)
• Subclinical hyperthyroidism is defined as: normal serum free T4 and T3 levels • Beta blockers (initially IV propranolol 5mg, then orally) unless contra-
with a TSH below normal range (usually <1mIU/L) indicated; diltiazem can be used if propranolol is contraindicated
• Common causes include multinodular goitre and excessive thyroxine • Hydrocortisone administration is also recommended
treatment • For severe agitation, sedate with chlorpromazine
• The importance in recognising subclinical hyperthyroidism lies in the • Keep cool with tepid sponging and paracetamol (avoid aspirin which can
potential effect on the cardiovascular system (can precipitate atrial increase T4 levels)
fibrillation) and bone metabolism (increase likelihood of osteoporosis); it • Patients who fail medical therapy should be treated with therapeutic plasma
may also impact on quality of life and increase the likelihood of dementia exchange or thyroidectomy
• TSH levels often revert to normal; therefore levels must be persistently low
to warrant intervention and often only monitoring is required; a reasonable
treatment option is a therapeutic trial of low-dose antithyroid agents for
approximately 6 months in an effort to induce a remission

166 / Chapter 5 Endocrinology: Hyperthyroidism notes Mind Maps for Medicine \ 167
 • Exogenous e.g. insulin and oral • Insulinoma, Immune • Although rare, insulinomas are the most common cause of endogenous Autonomic symptoms
hypoglycaemics (most common), hypoglycaemia (e.g. anti-insulin hyperinsulinaemia in adults
Hypoglycaemia is a lower than alcohol excess, ACE inhibitors, receptor antibodies in Hodgkin’s • Around 5–7% are malignant and around 7–10% occur in association • Sweating
normal level of blood glucose. It can salicylate poisoning disease), Infection e.g. malaria with MEN-1 • Anxiety
be defined as ‘mild’ if the episode is • Pituitary insufficiency • Non-pancreatic neoplasms • Diagnosis: low glucose (<2.2mmol/L) at the same time as inappropriately • Hunger
self-treated and ‘severe’ if assistance • Liver causes including severe liver (e.g. fibroma, sarcoma, hepatoma), high insulin and C-peptide; this is usually diagnosed with an overnight fast • Tremor
by a third party is required. Although failure, glycogen storage disease, Nesidioblastosis but in less clear-cut cases a prolonged fast of up to 72h may be carried out • Palpitations
definitions of hypoglycaemia galactosaemia • Starvation under observation in hospital • Dizziness
vary greatly, any blood glucose • Adrenal insufficiency, congenital • Insulinomas may be too small to be seen on CT scans and further
<4.0mmol/L should be treated. Adrenal hyperplasia, After investigation with endoscopic ultrasound should be considered Neuroglycopenic symptoms
bariatric surgery (‘Dumping
syndrome’)
Causes (EXPLAINS) • Surgical removal is the mainstay of treatment
• Confusion, change in behaviour
• Fatigue, drowsiness
Definition Insulinoma
•
•
Blurred vision
Weakness
• Dizziness
• Slurred speech
• Seizures
• Coma
Hypoglycaemia General malaise

• Headache
• Nausea

Management Clinical features

Acute Long-term

• Treat underlying cause

Acute management
• Better education and less tight
of hypoglycaemia
glycaemic control in diabetes, not
missing meals or stopping/reducing
CONSCIOUS UNCONSCIOUS hypoglycaemic medication
• Safety precautions e.g. with driving
(refer to DVLA guidelines)

Able to cooperate Unable to cooperate 1. ABCD approach

Diagnosis/investigations
Unable to swallow?
and swallow? but can swallow? 2. Stop insulin therapy

Confirming diagnosis (see Fig. 1)

• Fruit juice e.g. 200ml Glucogel/dextrogel:
IV access available? • Capillary glucose or
orange juice squeeze in mouth
• Plasma glucose <4mmol/L

cae ms
• Lucozade

Low ose le
glu
gly pto
a
mi
• Glucose tablets No Yes

c
Exploring underlying cause

blo vel
ypo sym

od
• 3–4 teaspoons of

of h s and
sugar dissolved in *Glucagon IM IV glucose e.g. 10% (usually not required if taking hypoglycaemic agents) Diagnosing

n
Sig
water e.g. 1mg dextrose over 15 min • HbA1c, LFTs, TFTs, U&Es, 0900h cortisol ± short Synacthen test hypoglycaemia
(if suspected adrenal insufficiency)
• Blood and urine assays for sulfonylureas: to detect factitious
hypoglycaemia caused by these drugs
• Plasma insulin, glucose and C-peptide: hypoglycaemic
Glucose >4mmol/L?
insulinaemia is present with insulinoma, sulfonylureas and
Relief of symptoms with
exogenous insulin administration, but C-peptide is only raised
increased blood glucose
Long-acting carbohydrate with endogenous insulin i.e. with insulinoma
Fig. 1 According to Whipple’s triad, the diagnosis
e.g. biscuits, sandwich, milk of hypoglycaemia rests on the 3 criteria shown

*Note: IM glucagon has a relatively slow onset of action and relies on glycogen stores; therefore it may not be suitable for those in starved states,
liver disease and in young children (IV glucose should be given instead)

168 / Chapter 5 Endocrinology: Hypoglycaemia Mind Maps for Medicine \ 169

 By severity Symptoms Signs
Notes
Hyponatraemia
• Mild hyponatraemia Serum Na +
• Usually asymptomatic • Decreased level of consciousness
130–135mmol/L • Anorexia • Cognitive impairment (e.g. short-
• Moderate hyponatraemia Serum • Nausea term memory loss, disorientation,
Na+ 125–129mmol/L • Headache confusion, depression)
• Severe hyponatraemia Serum Na+ • Lethargy • Focal or generalised seizures
<125mmol/L • Personality change • Signs of hypovolaemia e.g. dry
• Muscle cramps mucous membranes, tachycardia,
By rate of onset • Weakness prolonged capillary refill time
Hyponatraemia refers to a low • Confusion • Signs of hypervolaemia e.g. raised
concentration of sodium in the • Acute hyponatraemia Duration <48h • Ataxia JVP, pulmonary and peripheral
blood and is defined as a serum This depends on plasma osmolality • Chronic hyponatraemia Duration • Drowsiness oedema
sodium <136mmol/L. and fluid status (see Fig. 1) ≥48h

Clinical features
Definition Causes Classification

Hyponatraemia

Investigations
Complications

• Cerebral oedema Management Blood tests

• Cerebral herniation • U&Es To confirm hyponatraemia
• Death • TFTs Severe hypothyroidism can cause
• Central pontine myelinolysis: if The management of hyponatraemia depends on the • Hypertonic saline can be used in severe hyponatraemia
hyponatraemia corrected too fast fluid status and underlying cause; hyponatraemia hyponatraemia in ITU/HDU setting • Plasma osmolality (paired with urine
should be corrected slowly as if corrected too fast it • Vasopressin receptor antagonist, e.g. osmolality) (see Fig. 1)
can result in central pontine myelinolysis. tolvaptan, may be useful for SIADH; • Glucose Hyperglycaemia can cause
however, can induce thirst and can increase hyponatraemia
Hypovolaemia Na+ levels too rapidly • Cortisol (0900h) Adrenocortical deficiency can
• Stop underlying cause e.g. diuretics cause hyponatraemia
Hypervolaemia
• Treat with IV normal saline • Lipids and albumin Raised levels can result in
• Fluid restrict pseudohyponatraemia
Euvolaemia • Treat underlying cause, e.g. heart failure,
Urine
with loop diuretics
• Fluids restriction: 500–1000ml/day
• Demeclocycline: licensed to treat hyponatraemia • Urine osmolality (paired with plasma
associated with SIADH secondary to malignant osmolality)
disease where fluid restriction is ineffective and • Urinary sodium (see Fig. 1)
patient does not have cirrhosis
Imaging

• CXR (rule out infection/lung malignancy in

SIADH)
• CT chest, abdomen, pelvis (rule out
malignancy in SIADH)

170 / Chapter 5 Endocrinology: Hyponatraemia Mind Maps for Medicine \ 171

 Hyponatraemia notes Notes
Hyponatraemia

Syndrome of inappropriate ADH

secretion (SIADH)
Hyponatraemia causes Definition

SIADH is inappropriate ADH secretion from the posterior pituitary gland or from
an ectopic source despite low serum osmolality.
Plasma osmolality
Causes

• Neurological Tumour, meningoencephalitis, head trauma, infection,

Guillain–Barré syndrome, multiple sclerosis, SLE, subarachnoid or subdural
haemorrhage, sinus thrombosis, AIDS, porphyria
High osmolality Low osmolality Normal osmolality • Pulmonary Lung small cell carcinoma, mesothelioma, pneumonia,
cystic fibrosis
• Malignancies Lung small cell carcinoma, pancreas, prostate, thymus,
lymphoma
• Drugs e.g. SSRIs, opiates, thiazide diuretics, carbamazepine, tricyclic
Hyperglycaemia, Pseudohyponatraemia antidepressants, vincristine, cyclophosphamide, phenothiazines, oxytocin
Hypervolaemia Euvolaemia Hypovolaemia
mannitol, toxic alcohol from high protein/lipids
Diagnostic features

• Hyponatraemia
• Plasma hypo-osmolality proportional to hyponatraemia
• Inappropriately elevated urine osmolality
• Persistent urine Na+ >30mmol/L with normal salt intake
Urinary sodium Urinary sodium Urinary sodium Urinary sodium Urinary sodium Urinary sodium • Euvolaemia
>20 mmol/L <20 mmol/L >40 mmol/L <40 mmol/L >20 mmol/L <20 mmol/L • Normal thyroid and adrenal function

Management

Adrenocorticol • Fluid restriction

Heart failure, SIADH, Acute water load,
deficiency, renal Vomiting, • Treat underlying cause e.g. malignancy, infection
Renal failure/ cirrhosis, nephrotic glucocorticoid psychogenic
failure, diuretics, diarrhoea, • Hypertonic saline in acute severe case
disease syndrome, deficiency, severe polydipsia, tea and
cerebral salt skin loss • As above; demeclocycline and vasopressin receptor antagonists
primary polydipsia hypothyroidism toast/beer diets
wasting e.g. tolvaptan can also be used

Fig. 1 Causes of hyponatraemia according to plasma osmolality, fluid status and urinary sodium

172 / Chapter 5 Endocrinology: Hyponatraemia notes Mind Maps for Medicine \ 173
 • Pituitary tumours For example, adenomas • Empty sella syndrome Radiological diagnosis Presentation varies from asymptomatic to acute pituitary failure with acute • Men Loss of libido, impotence, mood impairment, loss of facial, scrotal
• Non-pituitary tumours Craniopharyngiomas, of absence of normal pituitary within the sella collapse and coma, depending on the aetiology, rapidity of onset, and and body hair; decreased muscle mass, osteoporosis
meningiomas, gliomas, chordomas, turcica; usually benign and asymptomatic but may predominant hormones involved. • Children Delayed puberty
ependymomas, metastases develop headaches and hypopituitarism • ACTH deficiency Fatigue, pallor, anorexia, weight loss, weakness, dizziness, • Growth hormone deficiency Short stature, decreased muscle mass and
• Infiltrative processes: Sarcoidosis, histiocytosis X, • Iatrogenic Irradiation, neurosurgery, withholding nausea, vomiting, circulatory collapse, shock strength, visceral obesity, fatigue, decreased quality of life, impairment of
haemochromatosis, lymphocytic hypophysitis previous chronic glucocorticoid replacement • TSH deficiency: attention and memory, dyslipidaemia, premature atherosclerosis, growth
• Infections Cerebral abscess, meningitis, • Traumatic head injury • Adults Tiredness, cold intolerance, constipation, hair loss, dry skin, restriction (children)
Hypopituitarism is the inability encephalitis, tuberculosis, syphilis • Congenital Kallmann syndrome (congenital hoarseness, weight gain, bradycardia and hypotension • Antidiuretic hormone deficiency Polyuria and polydipsia
of the pituitary gland to provide • Ischaemia and infarction Subarachnoid hypogonadotropic hypogonadism with anosmia) • Children Delayed development, growth restriction and intellectual
sufficient hormones usually due to haemorrhage, ischaemic stroke, Sheehan • Pituitary hypoplasia/aplasia impairment
pituitary disease or hypothalamic syndrome (postpartum haemorrhage with • Genetic cause e.g. HEXS1, LHX3, PIT1, PROP1 • Gonadotropin deficiency:
abnormalities associated with anterior pituitary infarction), pituitary apoplexy gene mutations, septo-optic dysplasia • Women Oligomenorrhoea, loss of libido, dyspareunia, breast atrophy,
reduced stimuli to hormonal (caused by an acute infarction of a pituitary • Idiopathic infertility, osteoporosis
secretion. adenoma)

Definition Causes Clinical features

Notes
Hypopituitarism

Hypopituitarism

Management Investigations

• Acute pituitary failure may require resuscitation, including IV fluids • Blood glucose, U&Es Disturbances of renal function,
• Surgical or medical removal of lesion if tumour is the underlying cause; glucose and electrolytes are common
removal of macroadenomas that do not respond to medical therapy • Hormone assays TFTs, prolactin, gonadotropins,
• In pituitary apoplexy, prompt surgical decompression may be life-saving testosterone and cortisol
• Hormone replacement as appropriate and treatment of the underlying • Measure gonadotropins, TSH, growth hormone,
cause: glucose and cortisol Following triple stimulation with
• Glucocorticoids (usually hydrocortisone but occasionally prednisolone gonadotropin-releasing hormone (GnRH), thyrotropin-
or dexamethasone) is required if the ACTH–adrenal axis is impaired, releasing hormone (TRH) and insulin-induced
especially in acute presentations; increased doses of glucocorticoids are hypoglycaemia
required following any form of emotional or physical stress (e.g. during an • Pituitary function tests:
infection) to prevent acute decompensation • For growth hormone deficiency: IGF-1 (low levels
• Secondary hypothyroidism: thyroid hormone replacement; in suggest growth hormone deficiency), insulin-stress test
panhypopituitarism is important to restore cortisol levels before thyroxine (measures growth hormone response to IV insulin)
• Gonadotropin deficiency: testosterone replacement in men and • For ACTH deficiency: Synacthen test (measures the
oestrogens (with or without progesterone) for women (combined oral adrenal response to ACTH), insulin-stress test (measuring
contraceptive pill for premenopausal women); gonadotropins or pulse ACTH response to insulin-induced hypoglycaemia)
gonadotropin-releasing hormone if fertility is required and the defect is • For TSH deficiency: TRH stimulation test
hypothalamic (with intact pituitary) (TSH response to IV-administered TRH)
• Growth hormone replacement for growth hormone deficiency • Cranial MRI To exclude tumours and other lesions of the
• Vasopressin replacement for diabetes insipidus sellar and parasellar region after hypopituitarism has been
confirmed

174 / Chapter 5 Endocrinology: Hypopituitarism Mind Maps for Medicine \ 175

 • 10–15% are malignant in nature Symptoms Signs
• The adrenal medulla is the location of the majority • The clinical manifestations of a
of the body’s chromaffin cells which produce phaeochromocytoma arise from • Headache • Hypertension
catecholamines excessive catecholamine secretion by • Approx. 70% are sporadic • Sweating • Postural hypotension
• It plays an important role in the fight-or-flight the tumour • Up to 30% are inherited as part of: • Palpitations • Tremor
response: • Catecholamines typically secreted are • Multiple endocrine neoplasia (MEN) • Tremor • Tachycardia
• Cardiovascular: ↑heart rate, ↑force of heart noradrenaline and adrenaline; but syndrome 2: occurs bilaterally in 70% of MEN • Nausea • Hyperglycaemia
Phaeochromocytoma is a some tumours produce dopamine syndromes
contraction, mainly vasoconstriction and • Weight loss • Hypertensive retinopathy
rare tumour that secretes • Excessive and uncontrolled • Neurofibromatosis type 1: incidence of
vasodilatation in the cardiac and skeletal muscle • Weakness • Pallor
catecholamines. It is catecholamine results in increased phaeochromocytoma is approx. 1%
• Metabolic and endocrine actions: • Anxiety • Fever
derived from chromaffin • von Hippel–Lindau (VHL) disease: 15–20%
↑glycogenolysis, ↑lipolysis, ↑glucagon secretion, stimulation of alpha- and beta- • Sense of doom
cells, usually in the adrenal develop phaeochromocytoma and approx. 50% are
↓insulin secretion, ↑renin secretion adrenergic receptors in various parts of • Epigastric/flank pain
medulla; however, occasionally the body bilateral
• Miscellaneous: dilation of pupils, contraction of
(18%) there are extra-adrenal
spleen, ejaculation, inhibition of micturition
phaeochromocytomas (called
paragangliomas). Pathophysiology Causes
Adrenaline physiology Clinical features
Definition

Phaeochromocytoma

Notes
Phaeochromocytoma
Prognosis
• For non-malignant
phaeochromocytoma the 5-year
survival rate is >95%
• For malignant
phaeochromocytoma the 5-year
survival rate is <50%
Hypertensive crisis

Management Investigations Any of the following may precipitate a hypertensive crisis:

• Induction of anaesthesia
• Opiates
• Associated MEN conditions must be searched for and, • Dopamine antagonists
if found, managed accordingly, including genetic Biochemistry • Decongestants such as pseudoephedrine
counselling • Drugs that inhibit the reuptake of catecholamines,
• Urine 24-h collection of total catecholamines,
• Surgical resection of the tumour is the treatment of including tricyclic antidepressants and cocaine
vanillylmandelic acid (VMA) and metanephrines
choice: • X-ray contrast media
• Bloods ↑Plasma catecholamines and plasma
• Preoperative treatment with alpha blockers • Childbirth
metanephrines; calcium and glucose may be raised
(phenoxybenzamine is started at least 7–10 days
before operation) and beta blockers (e.g. Imaging
propranolol) is required to control BP and prevent
intraoperative hypertensive crises To localise the tumour:
• A 24-h urine collection for total catecholamines,
• CT abdomen and pelvis ± mediastinum and neck
metanephrines and VMA must be checked 2 weeks
• MRI abdomen and pelvis ± mediastinum and neck
after operation
• Meta-iodobenzylguanidine (MIBG) scan
• Lifelong annual biochemical testing is recommended
• PET scan
to detect recurrent or metastatic disease

176 / Chapter 5 Endocrinology: Phaeochromocytoma Mind Maps for Medicine \ 177

 • The pathophysiology of PCOS is not completely • Menstrual disturbance: oligomenorrhoea or amenorrhoea • Sex hormones ↑Total testosterone or normal (because The Rotterdam criteria
Polycystic ovary syndrome (PCOS) understood but it is thought to be centred on • Infertility or subfertility SHBG is suppressed in PCOS), ↑free testosterone;
is a complex endocrine disorder hyperandrogenism • Acne biochemical evidence requires ↑free androgen index 1. Menstrual disturbance:
thought to affect 5–20% of women • Insulin resistance is an important part of • Hirsutism (see Fig. 1) (serum total testosterone/SHBG) Amenorrhoea (absent periods) or oligomenorrhoea (infrequent
of reproductive age, with clinical the syndrome that leads to hyperinsulinaemia • Alopecia • FSH, LH ↑LH with ↑LH:FSH ratio (>2) periods)
features that include hirsutism and causing insulin to stimulate LH receptors in • Obesity or difficulty losing weight • Prolactin May be ↑ but done mainly to exclude 2. Hyperandrogenism:
acne (due to excess androgens), the theca cells to further increase androgen • Psychological symptoms: mood swings, depression, anxiety, hyperprolactinaemia as a cause for symptoms Clinical hyperandrogenism (Ferriman–Gallwey score >8) or
oligomenorrhoea or amenorrhoea, secretion poor self-esteem • TSH Exclude thyroid dysfunction biochemical hyperandrogenism (elevated total/free testosterone)
and multiple cysts in the ovary. • This is compounded by central adiposity which • Sleep apnoea • OGTT/HbA1c ↑Risk of impaired glucose tolerance and 3. Polycystic ovaries:
contributes to reduced sex hormone binding • Acanthosis nigricans (brown to black, poorly defined, velvety diabetes in PCOS Polycystic ovaries on US (≥12 antral follicles in one ovary or ovarian
Note: Polycystic ovaries on US are
globulin (SHBG) thus increasing the free hyperpigmentation of the skin); it is associated with insulin • Lipids ↑Risk of dyslipidaemia in PCOS volume ≥10cm3) (see Fig. 2)
very common and can be seen in up
circulating androgen index resistance • Pelvic US The average volume is three times that
to 33% of women of reproductive age;
• Hyperandrogenism results in an increased of normal ovaries; however, the syndrome can exist
however, the majority of women with
number of growing follicles which without the presence of polycystic ovaries
polycystic ovaries do not have features
subsequently produce anti-Müllerian
of PCOS and do not require treatment.
hormone resulting in a lack of dominant
follicle selection and anovulation thus
contributing to menstrual disturbance
Investigations
Definition and subfertility

Pathophysiology

Fig. 1 Hirsutism
Fig. 2 Polycystic ovary

Clinical features
• Impaired glucose tolerance Diagnostic criteria
and T2DM
• Cardiovascular disease
• Dyslipidaemia
• Infertility
• Pregnancy complications Polycystic ovary syndrome
• Sleep apnoea
• Endometrial cancer Notes
Polycystic ovary syndrome

Complications

Management

General measures Hirsutism and acne Metabolic management Subfertility management

management
• Weight loss and • Weight loss and exercise • Clomiphene induces ovulation
exercise have been • Local therapy: physical methods • Metformin for impaired glucose and has been proven to improve
shown to improve fertility, (waxing, shaving, bleaching, plucking, tolerance pregnancy rates
psychological symptoms depilation and electrolysis), laser • Orlistat can help with weight loss • Metformin may be used instead
and metabolic features treatment, eflornithine cream in obese women with PCOS and of or together with clomiphene to
• COCP: to control menstrual • A COCP may be used to help manage may improve insulin sensitivity improve pregnancy rates
irregularity in women that hirsutism • Gonadotropins are 2nd-line
require contraception • Co-cyprindiol (ethinylestradiol and treatment for inducing ovulation in
cyproterone): licensed for treating those resistant to clomiphene
hirsutism and acne • Laparoscopic ovarian drilling
• Spironolactone, flutamide and is 2nd-line treatment for inducing
finasteride may be used under ovulation
specialist supervision

178 / Chapter 5 Endocrinology: Polycystic ovary syndrome Mind Maps for Medicine \ 179
 Chapter 6
06
Neurology

Bell’s palsy.......................................................................................................................................................................182
Delirium...........................................................................................................................................................................186
Epilepsy............................................................................................................................................................................190
Extradural haematoma (epidural haemorrhage)...................................................................................194
Guillain–Barré syndrome......................................................................................................................................196
Migraine and other causes of headache....................................................................................................198
Motor neurone disease..........................................................................................................................................202
Multiple sclerosis.......................................................................................................................................................204
Myasthenia gravis......................................................................................................................................................206
Neurofibromatosis....................................................................................................................................................208
Parkinsonism................................................................................................................................................................210
Polyneuropathies.......................................................................................................................................................214
Transient ischaemic attack...................................................................................................................................216
Stroke................................................................................................................................................................................218
Subarachnoid haemorrhage..............................................................................................................................222
Subdural haematoma.............................................................................................................................................224

Mind Maps for Medicine \ 181

 Rapid onset (<72h): Idiopathic Systemic disease
• Unilateral sagging of the
An acute, unilateral, partial or mouth • Bell’s palsy (most common) • Diabetes mellitus
complete paralysis of the face • Likely cause is ischaemic compression of • One-sided facial paralysis (see Fig. 1) • Sarcoidosis
due to idiopathic unilateral the facial nerve within the facial canal due (see Fig. 1) • Guillain–Barré syndrome (usually
lower motor neurone facial to inflammation • Drooling of saliva Infectious bilateral facial nerve palsy)
nerve palsy. It is the most • Inflammation is most likely caused by a • Overall, it is relatively uncommon with • Speech difficulty • Rheumatoid arthritis and Sjögren
common cause of facial nerve viral infection (with herpes simplex virus an incidence of approx. 20–30 people • Hyperacusis • Ramsay Hunt syndrome syndrome
palsy but it is often a diagnosis and varicella zoster virus being the likely per 100 000 each year • Altered taste (loss of taste • Lyme disease • Vasculitides
of exclusion as other important culprits) although the exact pathogenesis • Most commonly seen at age 15–60 years sensation in the anterior • Meningitis
diagnoses such as stroke, remains unclear • It is more common in people who are two-thirds of tongue) • TB ENT
infections, parotid tumours, • There may be a familial component diabetic, immunocompromised, obese, • Failure of eye closure: may • Other viruses: HIV, EBV, CMV
middle ear disease should be in recurrent cases, possibly due to hypertensive or have upper respiratory cause watery or dry eyes • Acoustic neuroma
excluded first. anatomical abnormality of the facial canal conditions, or in pregnant women Intracranial lesions • Otitis media
Fig. 1 Bell’s palsy showing right
facial paralysis when the patient • Parotid tumours
• Stroke • Cholesteatoma
Definition Pathophysiology Epidemiology/risk factors
was asked to smile
• Brain tumours
• Multiple sclerosis (MS) Trauma

Clinical features • Basal skull fracture

• Forceps delivery
• Post acupuncture haematoma
• Diving (barotrauma)

Causes of facial nerve palsy

Bell’s palsy
Notes
Bell’s palsy

Prognosis Management Investigations

Eye care • Antivirals such as aciclovir are NO LONGER

• Most people with Bell’s palsy begin to recommended The diagnosis of Bell’s palsy is clinical and investigations
recover, even without treatment, within • ↓Tear production and difficulty closing are not usually required but may be done to rule out other
2–3 weeks (approx. 85%); complete recovery the eye puts people at risk of corneal Physiotherapy causes or determine the severity of palsy:
usually occurs within 3–4 months dryness, ulceration and even blindness; • Serology Raised borrelia antibodies (in Lyme disease)
• If untreated, around 15% of patients have appropriate eye care is vital ‘Facial re-training’ to improve facial motor or varicella zoster virus antibodies (in Ramsay Hunt
permanent moderate to severe weakness • Prescription of artificial tears and eye function may help but evidence of its syndrome)
• Poor prognostic features include: lubricants should be considered effectiveness is lacking. • MRI brain To rule out stroke, space-occupying lesion
• Complete palsy or severe degeneration • Taping the eyelids shut at night is or MS
(on electrophysiology) Botulinum • Nerve conduction studies Predict delayed recovery
recommended
• No signs of recovery by 3 weeks • If the cornea remains exposed after by showing axonal degeneration
Botulinum toxin can augment facial
• Age >60 years attempting to close the eyelid, urgent • Schirmer’s tear test Shows reduced flow of tears on
symmetry.
• Severe pain referral to ophthalmology should be the side of the palsy
• Ramsay Hunt syndrome considered Surgery • Stapedial reflex (audiological test) Absent if stapedius
• Associated with hypertension, diabetes or muscle is affected
pregnancy Steroids • Facial nerve decompression is an option for
patients with facial palsy not responding to
• Should be prescribed for patients within medical treatment
72h of onset of Bell’s palsy • If residual paralysis after 6–9 months,
• Prednisolone 1mg/kg or 60mg OD for referral to plastic surgery should be
5 days then reducing by 10mg each day considered for surgical nerve grafting
for a further 5 days (10 days in total)

182 / Chapter 6 Neurology: Bell’s palsy Mind Maps for Medicine \ 183
 Bell’s palsy notes Notes
Bell’s palsy

Neuroanatomy of the facial nerve Ramsay Hunt syndrome

(7th cranial nerve) Definition
• The facial nerve is largely motor in function, supplying the muscles
of facial expression Ramsay Hunt syndrome (herpes zoster oticus) is caused by the reactivation of
• In addition it has two major branches which arise during its intracranial the varicella zoster virus in the geniculate ganglion of the 7th cranial nerve.
course through the facial canal of the petrous temporal bone:
Epidemiology
• The chorda tympani, which carries taste from the anterior
two-thirds of the tongue It most commonly occurs in elderly people
• Nerve to stapedius muscle, which has a damping effect to (over 60 years) although it can affect all ages.
protect the ear from loud noise
• Therefore damage to the facial nerve in the temporal bone Clinical features
(e.g. in Bell’s palsy) causes hyperacusis and taste disturbance to the
anterior two-thirds of the tongue • Auricular pain (often first feature)
• Within the parotid gland, the nerve terminates by splitting into five • Facial nerve palsy on affected side (as above)
extracranial branches which can be remembered by the mnemonic • Vesicular rash around the ear (see Fig. 3) Fig. 3 Vesicular rash in Ramsay
‘Two Zebras Bit My Coccyx’: Temporal, Zygomatic, Buccal, Mandibular • Vertigo and tinnitus Hunt syndrome
and Cervical
Management

Clinically distinguishing upper vs • Oral corticosteroids: prednisolone regime as above

lower motor neurone lesions that • Oral antivirals e.g. aciclovir
• Analgesia: paracetamol (± codeine) and NSAIDs are 1st line; tricyclic
cause facial weakness antidepressants, gabapentin, pregabalin, and opioids are other options
• It is vital to be able to distinguish clinically facial weakness caused • Eye care as above
by an upper (central) vs a lower (peripheral) motor neurone lesion
Prognosis
as this will significantly alter the management plan
• Upper motor neurone lesions, such as a stroke, cause contralateral face • Recovery of facial nerve function is less likely than in Bell’s palsy
weakness sparing the forehead, while lower motor neurone lesions, • The prognosis is excellent for younger and otherwise healthy patients
such as a facial nerve injury, typically cause weakness involving the • Elderly people have increased risk of post-herpetic neuralgia, bacterial
whole ipsilateral face (see Fig. 2) infections and scarring

Peripheral facial palsy Central facial palsy

Loss of forehead & Preservation of

brow movements forehead & brow
movements

Inability to close
eyes & drooping
of eyelids

Loss of nasolabial Loss of nasolabial

folds & drooping folds & drooping
of lower lip of lower lip

Fig. 2 The innervation to the muscles of the upper face originates on both sides of the brain, whereas the innervation to the muscles of the lower face is from the contralateral side of
the brain only; when the cortex is injured, e.g. in a stroke, there is weakness in the contralateral lower face only, therefore ‘forehead sparing’; when the facial nerve is injured, there is
weakness in the ipsilateral upper and lower face

184 / Chapter 6 Neurology: Bell’s palsy notes Mind Maps for Medicine \ 185
 • Hypoxia Respiratory failure, MI, cardiac failure, PE Acute and fluctuating course (often worse at night) DELIRIUM:
• Endocrine Hyperthyroidism, hypothyroidism, hyperglycaemia, • Disordered thinking: slowed irrational, incoherent thoughts
• Older age ≥65 years hypoglycaemia, Cushing syndrome • Euphoric, fearful, depressed or angry
• Dementia • Infection Pneumonia, UTI, encephalitis, meningitis • Language impaired: rambling speech, repetitive and
• Renal impairment • Stroke And other intracranial events disruptive
• Hypoactive (40%) Characterised by people
• Sensory impairment • Nutritional Thiamine deficiency, vitamin B12, nicotinic acid, folate • Illusions, delusions: transient persecutory or delusions of
who become withdrawn, quiet and sleepy;
• Recent surgery • Theatre (post-op) Anaesthetic, opioid analgesics + other complications misidentification
most common type of delirium but often goes
• Multiple comorbidities • Metabolic Hypoxia, electrolyte disturbance, hypoglycaemia, hepatic • Reversal of sleep–wake pattern: may be tired during the day
unrecognised
• Physical frailty impairment, renal impairment and hypervigilant at night
• Hyperactive (25%) Characterised by people who
• Male sex • Abdominal Faecal impaction, malnutrition, urinary retention, bladder • Inattention
An acute, transient, global organic have heightened arousal and can be restless,
• Previous episodes catheterisation • Unaware/disoriented: disoriented to time, place and person
disorder of CNS functioning resulting agitated or aggressive
• Severe illness • Alcohol Intoxication or withdrawal • Memory deficits
in impaired consciousness and • Mixed (35%) Demonstrate both hyperactive and
attention. There are different types hypoactive features • Drugs Benzodiazepines, opioids, anticholinergics, anti-parkinsonian
of delirium: hypoactive, hyperactive medications, steroids
and mixed. Risk factors
Classification Causes (HE ISN’T MAD) Clinical features
Definition

Several screening tools can be used to help

diagnosis delirium (see Notes):
Diagnostic criteria • Abbreviated mental test score (AMTS)
Delirium • Confusion assessment method (CAM)
• 4 As test (4AT)

Notes
Delirium
Management
Investigations
Treat underlying cause:

• Treat any infections Routine investigations

• Correct any electrolyte disturbances
• Stop any offending drugs • Urine dipstick ± culture To exclude UTI
• Laxatives for faecal impaction • Bloods FBC (infection), U&Es (electrolyte disturbance), LFTs
• Temporary catheterisation for urinary retention (alcoholism, liver disease), calcium (hypercalcaemia), glucose
• Give analgesia if pain suspected (hypo/hyperglycaemia), CRP (infection/inflammation), TFTs
(hyperthyroidism), vitamin B12, folate, ferritin (nutritional
Reassurance and reorientation deficiencies), blood culture (sepsis)
• ECG (cardiac abnormalities, acute coronary syndrome)
• Reassure patient to reduce anxiety and disorientation; patient should • CXR (pneumonia/heart failure)
be reminded of time, place, day and date regularly
Investigations based on history/examination
Provide appropriate environment
• Arterial blood gas (hypoxia)
• Quiet, well-lit side room; consistency in care and staff; reassuring • CT head (head injury, intracranial bleed, CVA)
nursing staff; encourage family and friends to attend; optimise • Lumbar puncture (meningitis, encephalitis)
sensory acuity e.g. glasses, well-lit room, orientation aids • EEG (epilepsy)

Manage disturbed, violent or distressing

behaviour

• Oral low-dose haloperidol (0.5–4mg) or olanzapine (2.5–10mg)

186 / Chapter 6 Neurology: Delirium Mind Maps for Medicine \ 187

 Delirium notes Notes
Delirium

Delirium vs dementia 4AT

Delirium Dementia 1. Alertness

This includes patients who may be markedly drowsy (e.g. difficult
Sleep–wake cycle Disrupted Usually normal to rouse and/or obviously sleepy during assessment) or agitated/
hyperactive. Observe the patient. If asleep, attempt to wake with speech
Attention Markedly reduced Normal/reduced or gentle touch on shoulder. Ask the patient to state their name and
address to assist rating.
Arousal Increased/decreased Usually normal
Normal (fully alert, but not agitated, throughout assessment) 0
Autonomic features Abnormal Normal
Mild sleepiness for <10sec after waking, then normal 0
Duration Hours to weeks Months to years
Clearly abnormal 4
Delusions Fleeting Complex 2. AMT4
Age, date of birth, place (name of the hospital or building), current year
Course Fluctuating Stable/slow/
progressive No mistakes 0

Conscious level Impaired Not impaired 1 mistake 1

Hallucinations Common Less common 2 or more mistakes/untestable 2

Onset Acute/subacute Chronic 3. Attention

Months of the year backwards
Psychomotor activity Usually abnormal Usually normal
Achieves 7 months or more correctly 0

Starts but scores <7 months/refuses to start 1

Confusion assessment method (CAM)
Untestable (cannot start because unwell, drowsy, inattentive) 2
The confusion assessment method (CAM) involves assessing a patient for 4
features; diagnosis involves the presence of 1 and 2 + either 3 or 4: 4. Acute change or fluctuating course
1. Acute onset and fluctuating course Evidence of significant change or fluctuation in: alertness, cognition,
2. Inattention other mental function (e.g. paranoia, hallucinations) arising over the
3. Disorganised thinking last 2 weeks and still evident in last 24h
4. Altered consciousness
No 0

Yes 4
Abbreviated mental test score (AMTS)
4 or above Possible delirium ± cognitive impairment
1. Age? (1)
1–3 Possible cognitive impairment
2. Time to the nearest hour? (1)
0 Delirium or cognitive impairment unlikely
3. Recall address at end: ‘42 West Street’ (1)
4. ‘What year it is?’ (1)
5. ‘Where are you right now?’ (1)
6. Identify two people (e.g. doctor, nurse) (1)
7. ‘What is your date of birth?’ (1)
8. ‘When did the Second World War end?’ (1)
9. ‘Who is the current monarch?’ (1)
10. Count backwards from 20 to 1 (1)
(<8 correct Cognitive impairment likely)

188 / Chapter 6 Neurology: Delirium notes Mind Maps for Medicine \ 189
Epilepsy is a neurological condition Focal seizures Generalised seizures • Idiopathic (most common) • A seizure results when a sudden imbalance occurs between the
characterised by recurrent seizures • Cerebrovascular disease: cerebral infarction, cerebral haemorrhage and excitatory and inhibitory forces within the network of cortical neurones
unprovoked by any immediately • Previously termed partial seizures • These involve both sides of the venous thrombosis in favour of a sudden-onset net excitation
identifiable cause. An epileptic • These start in a specific area, on one side of the brain brain at the onset • Head injury • This imbalance can result from an alteration at many levels of brain
seizure is the sudden transient • The level of awareness can be used to further classify • Consciousness is lost immediately • Post cranial surgery function, from genes and subcellular signalling cascades to widespread
attack of symptoms and signs due focal seizures: focal aware (previously termed ‘simple • Can be further subdivided into • CNS infections: meningitis or encephalitis neuronal circuits
to abnormal electrical activity in the partial’), focal impaired awareness (previously motor (e.g. tonic–clonic) and • Neurodegenerative diseases: Alzheimer’s and multi-infarct dementia • If the affected cortical network is in the visual cortex, the clinical
brain, leading to a disturbance of termed ‘complex partial’) and awareness unknown non-motor (e.g. absence) are risk factors for epilepsy manifestations are visual phenomena; other affected areas of primary
consciousness, behaviour, emotion, • Focal seizures can also be classified as being motor, • Specific types include tonic– • Autoimmune disease: e.g. anti-NMDA receptor encephalitis and cortex give rise to sensory, gustatory or motor manifestations; the psychic
motor function or sensation. non-motor (e.g. déjà vu, jamais vu) or having other clonic (grand mal), tonic, clonic, anti-LG11 encephalitis phenomenon of déjà vu occurs when the temporal lobe is involved
features such as aura absence (petit mal) and atonic • Brain neoplasm
• Genetic diseases: e.g. Dravet syndrome
• Drugs: e.g. phenothiazines, isoniazid, tricyclic antidepressants, Pathophysiology
benzodiazepines, binge alcohol drinking or alcohol withdrawal
Definition Classification • Metabolic medical disorders: uraemia, hypoglycaemia, hyponatraemia,
hypernatraemia, hypercalcaemia and hypocalcaemia

Causes
• Injuries sustained during seizures
• Social stigmatisation and occupational issues
• Anxiety/depression
• Status epilepticus
• Sudden unexplained death in epilepsy (SUDEP)
Complications • Increased mortality rate from SUDEP, deaths due to
accidents during seizures, deaths due to status epilepticus
Epilepsy

Clinical features
Management Investigations
• Aura Subjective symptoms at the start of the seizure
• Bloods e.g. Glucose, Ca2+, LFTs to identify potential causes (the patient is aware of this) – suggestive of focal
General advice Neurosurgical treatment • EEG Supports the diagnosis of epilepsy and may be used to help to epilepsy e.g. strange feeling in the gut, déjà vu, strange
determine seizure type and epilepsy syndrome; it is however often normal smells or flashing lights
• Take precautions e.g. avoid swimming alone, avoid dangerous sports, e.g. • Neurosurgical treatment has particular benefit for selected people
in between attacks (therefore normal EEG does not rule out epilepsy) but • Potential triggers Sleep deprivation, stress, light
rock climbing, leave door open when having a bath with refractory focal epilepsy
during a seizure it almost always shows an abnormal pattern (typically sensitivity or alcohol use
• Driving: • Some neurosurgical procedures involve resection of part of the brain
showing a cortical spike or generalised spike activity); long-term video or • Specific features of the seizure:
• All patients must not drive and must inform the DVLA and the aim is to obtain complete seizure freedom
ambulatory EEG may be used if diagnostic uncertainty remains after clinical • Tonic Short-lived, abrupt, generalised muscle
• For first unprovoked seizure: 6 months off if there are no relevant • For the most commonly performed procedures, involving anterior
assessment and standard EEG stiffening (may cause a fall) with rapid recovery –
structural abnormalities on brain imaging and no definite epileptiform and medial temporal lobe resection, about 70% of patients will
• ECG In all those with altered consciousness, particularly those in older age suggestive of tonic seizure
activity on EEG (if not met then this is increased to 12 months) become seizure-free
groups, when disorders of cardiac rhythm may simulate epilepsy; 24-h • Generalised tonic–clonic Generalised stiffening
• For patients with established epilepsy or those with multiple unprovoked
ambulatory ECG and other cardiovascular tests e.g. implantable loop devices and subsequent rhythmic jerking of the limbs,
seizures: may qualify for a driving licence if they have been free from any
may also be helpful urinary incontinence and tongue biting
seizure for 12 months
• Neuroimaging (used to identify structural abnormalities): • Absence seizure Brief pauses, e.g. suddenly stop
Anti-epileptics (see Table 1) • MRI brain Imaging investigation of choice and particularly important in talking full sentence then carrying on where left off
those with a focal onset on history (unless examination or EEG suggests (presents in childhood)
Anti-epileptics are usually started following a second epileptic seizure; NICE evidence of benign focal epilepsy), and in those who do not respond to • Atonic seizure Sudden onset of loss of muscle tone
guidelines suggest starting anti-epileptics after the first seizure if any of the 1st-line medication causing falls
following are present: • CT brain To identify gross pathology if MRI is not available or is • Myoclonic seizure Brief, ‘shock-like’ involuntary
• the patient has a neurological deficit contraindicated single or multiple jerks
• brain imaging shows a structural abnormality • Polysomnography May be used to confirm a diagnosis of sleep-related • Post-ictal phenomena (residual symptoms after
• the EEG shows unequivocal epileptic activity epilepsy the attack) e.g. Drowsiness, headaches, amnesia or
• the patient or their family or carers consider the risk of having a further • Handheld video recordings Asking family members or friends to video confusion (usually occur only after generalised tonic
seizure unacceptable record events should be considered in patients with uncertain diagnosis and/or clonic seizures).
(after consent from patient)
190 / Chapter 6 Neurology: Epilepsy Mind Maps for Medicine \ 191
 Epilepsy notes

Anti-epileptics Status epilepticus (Fig. 1)

Table 1 Anti-epileptics Convulsive status epilepticus is defined as a convulsive seizure which continues for a prolonged period (e.g. longer than 5 min), or when convulsive
seizures occur one after the other with no recovery between. Convulsive status epilepticus is an emergency and requires immediate medical attention
Sodium • Indication 1st-line treatment for patients with generalised seizures including generalised tonic–clonic, absence and
valproate myoclonic seizures Management
• Mechanism of action Blockage of voltage-gated Na+ channels and increased brain levels of gamma-aminobutyric acid
(GABA)
• Side effects Include nausea/vomiting, weight gain, hair loss, confusion, drowsiness, hepatotoxicity, thrombocytopenia,
teratogenicity, encephalopathy, oedema, SIADH Secure airway, high-flow oxygen
• Cautions/contraindications Pregnancy, acute porphyrias, known or suspected mitochondrial disorders, liver failure,
urea cycle disorders

Carbamazepine • Indications 1st-line treatment for focal seizures Secure IV access in a large vein and take venous blood gas and venous bloods
• Mechanism of action Preferentially binds to voltage-gated Na+ channels in their inactive form for glucose, electrolytes, calcium, U&Es, LFTs and toxicology screen and
• Side effects Rash, vomiting, drowsiness, hyponatraemia, leucopenia, thrombocytopenia, vision disturbance, movement anticonvulsant levels (if indicated)
disorders
• Cautions/contraindications Pregnancy, acute porphyrias, AV conduction abnormalities, bone marrow depression

Lamotrigine • Indications 1st-line treatment for focal seizures and 2nd-line for generalised tonic–clonic seizures Monitor ECG, oxygen saturations, temperature,
• Mechanism of action Blocks Na+ channels and suppresses the release of glutamate and aspartate pulse rate and blood pressure
• Side effects Aggression, agitation, diarrhoea, dizziness, drowsiness, sleep disorders, tremor, vomiting, aplastic anaemia
• Cautions/contraindications Myoclonic seizures (may be exacerbated), Parkinson’s disease (may be exacerbated),
caution in hepatic and renal impairment
IV dextrose if hypoglycaemia suspected/confirmed;
Levetiracetam • Indications 2nd-line treatment for patients with focal seizures
IV thiamine if alcoholism/malnourishment suspected
• Mechanism of action Exact mechanism unclear but binding to synaptic vesicle protein 2A (SV2A) appears to be the key driver
• Side effects Depression/anxiety, diarrhoea/vomiting, dyspepsia, insomnia, vertigo, blood dyscrasias
• Cautions/contraindications Caution in severe hepatic impairment and dose adjustment in renal impairment
IV lorazepam up to 4mg as 1st-line treatment; buccal midazolam
Ethosuximide • Indications 1st-line treatment for people with absent seizures 10mg or rectal diazepam 10–20mg should be given if
• Mechanism of action Binds to T-type voltage-sensitive calcium channels there is no IV access
• Side effects Aggression, agranulocytosis, reduced appetite, concentration impaired, generalised tonic–clonic seizure,
headache, bone marrow disorders
• Cautions/contraindications Acute porphyrias, pregnancy, caution in hepatic failure and renal failure
If seizures continue, contact ITU; IV phenobarbital or phenytoin is 2nd-line
Phenytoin • Indications Used for generalised, focal seizures and status epilepticus but not used 1st line in generalised and focal treatment; fosphenytoin (a prodrug of phenytoin) can be given more rapidly
seizures due to side effects and narrow therapeutic index and causes fewer injection-site reactions than phenytoin
• Mechanism of action Blocks voltage-dependent Na+ channels
• Side effects Gingival hyperplasia, hirsutism, coarsening of facial features, drowsiness, megaloblastic anaemia, peripheral
neuropathy, lymphadenopathy, dyskinesia, teratogenicity; toxicity causes dizziness, diplopia, nystagmus, slurred speech, For refractory convulsive status epilepticus summon anaesthetist and
ataxia, confusion, seizures ITU; administer IV midazolam, propofol or thiopental sodium for general
• Monitoring Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose, should anaesthesia with intubation and ventilation
be checked if: adjustment of phenytoin dose, suspected toxicity or detection of non-adherence to the prescribed
medication Fig. 1 Management of status epilepticus
• Contraindications Pregnancy, second- and third-degree heart block, sino-atrial block, sinus bradycardia, Stokes–Adams
syndrome; caution in hepatic impairment

Phenobarbital • Indications All seizures (including status epilepticus) except absent seizures
• Mechanism of action Acts on GABAA receptors enhancing synaptic inhibition
• Side effects Rash, sedation, bone disorders, depression, ataxia
• Cautions/contraindications Pregnancy, history of porphyria, severe hepatic impairment, caution in renal impairment

192 / Chapter 6 Neurology: Epilepsy notes Mind Maps for Medicine \ 193
 • Most commonly caused by a fractured • History of trauma Typically a head strike from sports injury or motor
temporal or parietal bone which causes vehicle accident; classically, this is followed by a lucid interval after which Notes
Extradural haematoma is the collection Parietal Frontal Extradural Acute
haematoma
respiratory
(epidural
distress
haemorrhage)
syndrome
damage to the middle meningeal artery or the patient deteriorates
of blood between the dura and the bone vein • Loss of consciousness
Sphenoid
(usually skull but may be spinal column) • Typically caused by trauma to the pterion, the • Severe headache
(see Fig. 1). It is immediately life-threatening. Ethmoid region where the frontal, parietal, temporal and • Nausea/vomiting
Temporal
Nasal sphenoid bones join together (see Fig. 2). • Confusion
Lacrimal
Occipital • Young people are more commonly • Evidence of skull fractures, haematomas or lacerations
Maxilla
Zygomatic
affected, not only because of the prevalent • Otorrhoea or rhinorrhoea Resulting from skull fracture
Skull demographics of patients with a head injury, • Unequal pupils
Mandible but it also relates to the changes that occur in • Hemiparesis With brisk reflexes and up-going plantar reflex
Dura
the dura in older patients, as the dura is much • Other focal neurological deficits e.g. Aphasia, visual field defects,
Extradural Fig. 2 The pterion, the weakest part of the skull; a more adherent to the inner surface of the skull numbness, ataxia
haematoma traumatic blow to the pterion may rupture the middle • Children are less likely to have associated skull • Bradycardia and hypertension Late signs (Cushing reflex)
meningeal artery causing an epidural haematoma fractures than adults • Death Follows a period of coma and is due to respiratory arrest
• Extradural haemorrhage in the spinal column • Spinal cord compression Produced by a haematoma in the spinal
Fig. 1 Diagram of an extradural haematoma can occur as a result of epidural anaesthesia or column; features may include: weakness, numbness, alteration in reflexes,
Pathophysiology lumbar puncture urinary incontinence and faecal incontinence

Definition Clinical features

Extradural haematoma
(epidural haemorrhage)

Investigations

• CT head Shows haematoma

(often biconvex /lens-shaped, see
Complications Management Prognosis Fig. 3); if there is deterioration, CT
must be repeated
• Bloods FBC, U&Es, coagulation
• Neurological deficits (temporary • ABC approach of resuscitation • Overall mortality rate is approx. 30% screen (if any suspicion of
or permanent) • Control of airway in unconscious • Those alert on admission rarely die abnormality of coagulation),
• Death patient; oxygen may be given but a low Glasgow Coma Scale (GCS) group and save
• Post-traumatic seizures • IV fluids may be required to score worsens the prognosis • Plain skull X-ray head May
• Post-concussion syndrome maintain the circulation and • The earlier the intervention, the more be normal or show fracture line
• Spasticity, neuropathic pain and preserve cerebral perfusion likelihood of survival crossing the course of the middle
urinary complications (spinal • Stabilise and transfer urgently • Risk factors for poor prognosis include: meningeal vessels
extradural haematoma) (with skilled medical and nursing older age, intradural lesions, increasing • Cervical spine X-ray Spinal injury
escorts) to a neurosurgical unit volume of haematoma, temporal must be excluded Fig. 3 Extradural haematoma on
• Measures to decrease raised ICP location, rapid clinical progression, • MRI head Gives detailed images CT scan
with IV mannitol or hypertonic pupillary abnormalities, raised ICP but may not be suitable for a
saline patient in an unstable condition
• Conservative management for
small haematomas
• Surgical intervention with burr
holes may be required to evacuate
a large haematoma

194 / Chapter 6 Neurology: Extradural haematoma (epidural haemorrhage) Mind Maps for Medicine \ 195
 • History of gastrointestinal or • GBS is usually triggered by an infection: Campylobacter • Weakness: • Reflexes May be reduced or absent
respiratory infection from 1 to jejuni, Epstein–Barr virus (EBV) and cytomegalovirus have all • In 60% of cases, onset occurs approximately 3 weeks • Sensory symptoms These can include paraesthesia
3 weeks prior to the onset of been linked after an infection and sensory loss, starting in the lower extremities
weakness • It is thought that the infectious organism shares epitopes • Usually presents with an ascending pattern of progressive • Autonomic symptoms Involvement of the autonomic
• Zika virus with an antigen in the peripheral nervous system leading to symmetrical weakness, starting in the lower extremities system may present, with reduced sweating, reduced
Guillain–Barré syndrome (GBS) • Vaccinations: live and dead vaccines autoantibody-mediated cell damage • This reaches a level of maximum severity 2 weeks after heat tolerance, paralytic ileus and urinary hesitancy;
describes an immune-mediated have been implicated • The suppressor T-cell response is reduced, suggesting a cell- initial onset of symptoms and usually stops progressing severe autonomic dysfunction may occur
demyelination of the peripheral • Malignancy: e.g. lymphomas, mediated immunological reaction directed at the peripheral after 5 weeks • Miller Fisher syndrome (variant of GBS):
nervous system often triggered especially Hodgkin’s disease nerves; occasionally, serum antibodies to myelin components • Facial weakness, dysphasia, diplopia or dysarthria may • Associated with ophthalmoplegia, areflexia and ataxia
by an infection. It can lead to • Pregnancy: incidence decreases are detected; nerve damage occurs segmentally; lymphocytes develop • Usually presents as a descending paralysis rather
life-threatening respiratory during pregnancy but increases in infiltrate the nerve roots and release cytotoxic substances that • In severe cases, muscle weakness may lead to than ascending as seen in other forms of GBS
failure. the months after delivery damage the Schwann cells and myelin respiratory failure • Anti-GQ1b antibodies are present in 90% of cases
• Correlation between anti-ganglioside antibody (e.g. anti-GM1) • Pain Neuropathic pain may develop, particularly in the legs;
and clinical features has been demonstrated; anti-GM1 back pain may also occur
Definition Risk factors antibodies are present in approx. 25% of patients

Clinical features
Pathophysiology

Guillain–Barré syndrome
Notes
Guillain–Barré syndrome

Complications Management Investigations

• Persistent paralysis • Plasma exchange Leads to shorter • Lumbar puncture Most patients have an elevated
• Respiratory failure requiring periods of ventilation and a shorter level of CSF protein, with no elevation in CSF cell
mechanical ventilation period until patients are able to walk counts (the gamma-globulin fraction is usually
• Aspiration pneumonia unaided raised); note: the rise in the CSF protein may not be
• Hypotension or hypertension • IV immunoglobulin If started within seen until 1–2 weeks after the onset of weakness
• Thromboembolism, pneumonia, 2 weeks from the onset of illness • Antibody screen In Miller Fisher syndrome there
skin breakdown it accelerates recovery as much as are often antibodies against GQ1b
• Cardiac arrhythmia plasma exchange • Spirometry Forced vital capacity is a major
• Urinary retention • DVT prophylaxis DVT due to determinant of the need for admission to ICU
• Ileus immobility; prevent with gradient and then the need for intubation
• Psychiatric problems e.g. depression, compression stockings and • Nerve conduction studies Abnormal in 85% of
anxiety subcutaneous low molecular weight patients, even early on in the disease; they show
heparin prolonged conduction velocities; repeat after
• Admission to ICU Intubation and 2 weeks if initially normal
assisted ventilation may be required • ECG Many different abnormalities may be seen:
• Pain relief May be required for 2nd-degree and 3rd-degree AV block, T-wave
neuropathic pain abnormalities, ST depression, QRS widening and
a variety of rhythm disturbances
• Campylobacter serology Should be performed;
positive titres identify a group with a poorer
prognosis

196 / Chapter 6 Neurology: Guillain–Barré syndrome Mind Maps for Medicine \ 197
Migraine is a primary headache • Tiredness, stress • Severe, often unilateral, throbbing headache
disorder which is characterised by • Alcohol • Aura: ‘classic’ migraine attacks are precipitated Notes
Migraine
episodic severe headaches (often • Combined oral contraceptive pill by an aura; these occur in around one-third of
but not always unilateral), with • Lack of food or dehydration migraine patients; typical aura are visual, progressive,
commonly associated symptoms • Cheese, chocolate, red wines, citrus fruits last 5–60min and are characterised by transient
such as photophobia, phonophobia • Menstruation hemianopic disturbance or a spreading scintillating
and nausea/vomiting. • Bright lights scotoma
• Nausea/vomiting
• Photophobia
Definition Common triggers • Phonophobia
• Attacks may last up to 72h

Clinical features

Migraine

Management Diagnostic criteria

International Headache Society

Acute
The diagnosis of migraine is a clinical diagnosis.
• Paracetamol e.g. 1g oral QDS; 1st-line treatment for pregnant
women as considered safe Table 1 International Headache Society diagnostic criteria
• NSAIDs e.g. Soluble aspirin 600–900mg (not in children)
or ibuprofen 400–600mg Point Criteria
• Triptans e.g. Sumatriptan; should be taken as soon as possible
after the onset of headache; oral, orodispersible, nasal spray and SC A At least 5 attacks fulfilling criteria B–D
injections are available
• Anti-emetics For nausea/vomiting e.g. buccal prochlorperazine or B Headache attacks lasting 4–72h (untreated or unsuccessfully
metoclopramide treated)
• Opiate-containing medication, e.g. codeine, should be avoided
C Headache has at least 2 of the following characteristics:
Prevention • Unilateral location
• Pulsating quality (i.e. varying with the heartbeat)
• Avoid triggers (if possible) • Moderate or severe pain intensity
• Prophylaxis should be given if patients are experiencing 2 or more • Aggravation by or causing avoidance of routine physical
attacks per month activity (e.g. walking or climbing stairs)
• Topiramate or propranolol 1st-line for prophylaxis according to
the person’s preference, comorbidities and risk of adverse events; D During headache at least one of the following:
propranolol should be used in preference to topiramate in women • Nausea and/or vomiting
of childbearing age as topiramate may be teratogenic and can • Photophobia and phonophobia
reduce the effectiveness of hormonal contraceptives
• Acupuncture If above measures fail, NICE recommend a course of E Not attributed to another disorder (history and examination
up to 10 sessions of acupuncture over 5–8 weeks or gabapentin do not suggest a secondary headache disorder or, if they do, it
• Riboflavin May be effective in reducing migraine frequency and is ruled out by appropriate investigations or headache attacks
intensity for some people do not occur for the first time in close temporal relation to the
• Triptans Frovatriptan or zolmitriptan can be used as a type of other disorder)
‘mini-prophylaxis’ for women with predictable menstrual migraine

198 / Chapter 6 Neurology: Migraine Mind Maps for Medicine \ 199

 Migraine notes

Table 2 Causes of headaches Table 2 (continued)

Condition Notes Condition Notes

Tension headache • Recurrent, non-disabling, bilateral headache, often described as a ‘tight band’ Subarachnoid • Sudden onset severe ‘thunderclap’ headache
• Not aggravated by routine activities of daily living haemorrhage • Nausea and vomiting
• Not associated with aura, nausea/vomiting or aggravated by routine physical activity • Meningism (photophobia, neck stiffness)
• May be associated with stress • See Ch6: Subarachnoid haemorrhage for further information
• Acute treatment: aspirin, paracetamol or an NSAID is 1st-line
• Prophylaxis: NICE recommend up to 10 sessions of acupuncture over 5–8 weeks; low-dose Head injury • Headache is common at the site of head injury but it may also be more generalised
amitriptyline is widely used in the UK for prophylaxis against tension-type headache • Serious head injury can lead to intracranial bleeds; a CT scan should be done if subdural or extradural
Chronic headache

haematoma is suspected
Medication overuse • Present for ≥15 days per month • See Ch6: Subdural haematoma and Extradural haematoma for further information
headache • Developed or worsened while taking regular symptomatic medication specifically for headaches
• Most common offending drugs are opioids and triptans Sinusitis • Facial pain: typically frontal pressure pain which is worse on bending forward
• There may be a psychiatric comorbidity • Associated symptoms include nasal discharge and nasal obstruction
• Simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches) • Postnasal drip may produce chronic cough

Acute single episode

• Opioid analgesics should be gradually withdrawn • Acute sinusitis can be treated with analgesia, intranasal decongestants and antibiotics for severe
presentations; intranasal corticosteroids are often useful for recurrent or chronic sinusitis
Raised intracranial • e.g. Tumour, idiopathic intracranial hypertension
pressure • Typically worse on waking, lying down or bending forward, or with coughing Acute glaucoma • Severe pain which may be ocular or headache
• Associated with vomiting, papilloedema, fits and neurological signs • Decreased visual acuity, red eye, haloes, semi-dilated non-reacting pupil, hazy cornea
• CT or MRI scan is investigation of choice • Symptoms worse with mydriasis e.g. watching TV in a dark room
• Lumbar puncture contraindicated until after imaging • Systemic upset may be seen, such as nausea and vomiting and even abdominal pain
• Requires urgent ophthalmology assessment
Migraine See Mind Map
Meningitis/encephalitis • Meningitis: classically presents with fever, photophobia, neck stiffness, headache, purpuric rash
Recurrent acute attacks of headache

Cluster headache • Intense pain around one eye; recurrent attacks ‘always’ affect same side • Encephalitis: classically presents with headache, fever, confusion/odd behaviour, seizures, reduced
• Patient is often restless during an attack due to severity of pain consciousness
• Pain typically occurs once or twice a day, each episode lasting 15 min to 2 hours with clusters typically • Requires CT scan followed by lumbar puncture for diagnosis (in absence of raised intracranial
lasting 4–12 weeks pressure)
• Associated eye symptoms include redness, lacrimation, lid swelling
• More common in men and smokers Central venous sinus • Headache which may be sudden onset
• Acute: 100% oxygen, subcutaneous triptan thrombosis • There may be associated nausea and vomiting, cranial nerve palsies, vision disturbance, seizures
• Prophylaxis: verapamil is 1st-line; a tapering dose of prednisolone can also be considered • The diagnosis is usually made with the aid of CT or MRI scan
• Specific treatment involves anticoagulation or thrombolytic treatments
Trigeminal neuralgia • A unilateral disorder characterised by transient electric shock-like pains, abrupt in onset and
termination, limited to one or more divisions of the trigeminal nerve
• The pain is commonly triggered by light touch e.g. washing, shaving, talking and brushing the teeth, Red flags for headaches
and frequently occurs spontaneously
• Immunocompromised e.g. HIV or on immunosuppressive drugs • Change in personality
• Carbamazepine is 1st-line treatment
• Age <20 years and a history of malignancy • Impaired level of consciousness
• History of malignancy known to metastasise to the brain • Headache exacerbated by cough, Valsalva (trying to breathe out with
Giant cell arteritis (GCA) • Typically patient >60 years old
Subacute onset

• Sudden-onset headache reaching maximum intensity within 5min nose and mouth blocked), sneeze or exercise
• Usually rapid onset (e.g. <1 month) of unilateral headache
• Vomiting without other obvious cause • Orthostatic headache (headache that changes with posture)
• Often associated jaw or tongue claudication
• Recent (typically within the past 3 months) head trauma • Symptoms suggestive of GCA (see Table 2)
• Tender, palpable temporal artery
• Worsening headache with fever • Symptoms suggestive of acute narrow-angle glaucoma (see Table 2)
• Raised ESR
• New-onset neurological deficit • A substantial change in the characteristics of patient’s headache
• See Ch7: Giant cell arteritis for further information
• New-onset cognitive dysfunction

200 / Chapter 6 Neurology: Migraine notes Mind Maps for Medicine \ 201
Motor neurone disease (MND) is a • MND is a degenerative condition that affects motor
rare but devastating neurological neurones, specifically the anterior horn cells of the spinal Notes
Motor neurone disease
condition of unknown cause which cord and the motor cranial nuclei
can present with both upper and • The cause of the disease is unknown, although 5% of • MND is relatively uncommon
lower motor neurone signs. It leads those affected have a familial form of the disease due to a with annual incidence of
to progressive paralysis and eventual mutation in the superoxide dismutase-1 gene around 2 cases per 100 000
death from respiratory failure. • It may be caused by abnormality of mitochondrial function population
causing oxidative stress in motor neurones for which there • It can occur at any age but
may be several causes is more common in people
Definition • It results in lower motor neurone (LMN) and upper motor aged >50 years
neurone (UMN) dysfunction, leading to a mixed picture of • The male to female ratio is 2:1
muscular paralysis, typically with LMN signs predominating • Approximately 5–10% of
cases are inherited

Pathophysiology Epidemiology

• Prognosis is poor with 50%

of patients dying within
3 years
• Most patients die of
respiratory failure

Prognosis Motor neurone disease

Clinical features/types
Management
• Amyotrophic lateral sclerosis (ALS)
The most common form of MND; it is
Riluzole Investigations a combination of disease of the lateral
corticospinal tracts and anterior horn cells
• A neuroprotective glutamate-release inhibitor; There are no specific investigations producing a progressive spastic tetraparesis
the only drug of proven disease-modifying efficacy that will confirm a diagnosis of MND; and paraparesis with added lower motor
• It is used mainly in ALS a range of investigations are carried neurone signs (muscle wasting and
• Prolongs life by approximately 3 months out to confirm consistent features and fasciculation)
exclude other possible pathologies: • Progressive bulbar palsy and
Other symptomatic treatment pseudobulbar palsy Approximately 20%
• Nerve conduction studies Show
normal motor conduction and can of people with MND have this type; it
• Dysarthria Speech assessment and communication aids
help exclude a neuropathy results from the destruction of the upper
• Dysphagia Feeding gastrostomy; cricopharyngeal
• EMG Shows a reduced number of (pseudobulbar) and lower (bulbar palsy)
myotomy
action potentials with an increased motor neurones in the lower cranial nerves;
• Dysphonia Speech therapists can give expert advice on
amplitude this results in dysarthria, dysphagia with
speech and swallowing difficulties
• MRI Usually performed to exclude the wasting, and fasciculation of the tongue
• Saliva problems Consider a trial of antimuscarinic
differential diagnosis of cervical cord • Progressive muscular atrophy An
medicine as the 1st-line treatment for sialorrhoea in
compression and myelopathy uncommon form of MND; predominantly
people with MND
lower motor neurone lesion of the spinal cord;
• Muscle weakness Physiotherapy, walking aids, splints
the small muscles of the hands and feet are
• Muscle cramps Consider quinine as 1st-line and
usually first affected, but muscle spasticity is
baclofen as 2nd-line treatment
absent
• Muscle stiffness, spasticity or increased tone
• Primary lateral sclerosis Another rare type
Consider baclofen, tizanidine, dantrolene or gabapentin
of MND; it mainly causes weakness in the leg
• Non-invasive ventilation (usually BIPAP) Used at
muscles and there is progressive tetraparesis
night; studies have shown a survival benefit of around
7 months

202 / Chapter 6 Neurology: Motor neurone disease Mind Maps for Medicine \ 203
Multiple sclerosis (MS) is an • The cause of MS is not completely understood spinal cord (see Fig. 1); in this way movement and Relapsing–remitting disease Visual
acquired, chronic, cell-mediated but it is thought to be caused by both genetic and sensation may be impaired
autoimmune condition environmental factors • The lesions are disseminated in both ‘space and time’ • Most common form (in around 85% of • Optic neuritis: common presenting
characterised by multiple • It is thought to be an autoimmune disease in which i.e. episodes occur months or years apart and affect patients) feature; usually unilateral
plaques of demyelination in exposure of a specific infectious agent e.g. EBV in early different anatomic locations • Acute attacks (e.g. last 1–2 months) • Optic atrophy
the central nervous system life may predispose to the later development of MS in a Healthy followed by periods of remission and • Internuclear ophthalmoplegia
that can affect the brain, genetically susceptible host periods of stability • Uhthoff’s phenomenon: worsening of
Schwann cells
brainstem and spinal cord. • The non-self antigen mimics proteins in myelin; this Node of Ranvier vision following rise in body temperature
• 3 times more common in women
antigen is presented on the surface of macrophages Secondary progressive disease
• Most commonly diagnosed in people
in combination with class 2 MHC causing antibodies Sensory
Definition produced by B cells and T cells to attack CNS myelin Neurone affected by MS
aged 20–40 years • Describes relapsing-remitting patients who
• It is the most common cause of have deteriorated and have developed • Pins/needles
because of molecular mimicry Myelin
Fibre exposed
damaged neurological disability in young adults neurological signs and symptoms between • Numbness
• This results in inflammation, demyelination and axonal
loss leading to plaque formation; this slows or blocks relapses • Trigeminal neuralgia
the transmission of signals to and from the brain and Epidemiology • Approximately 65% of patients with
relapsing-remitting disease go on to
• Lhermitte’s sign: paraesthesia in limbs on
neck flexion
Fig. 1 Healthy neurone vs neurone in MS
develop secondary progressive disease
Good prognosis features: within 15 years of diagnosis Motor
• Female sex • Gait and bladder disorders are typically seen
• Young age of onset (i.e. 20s or 30s) Pathophysiology • Spastic weakness: most commonly seen
• Relapsing-remitting disease Primary progressive disease in the legs
• Sensory symptoms only
• Accounts for 10% of patients Cerebellar
• Long interval between first two
relapses • Progressive deterioration from onset
• More common in older individuals • Ataxia
• Complete recovery between relapses • Tremor
• Dysarthria
Prognosis Multiple sclerosis Types
• Vertigo

ENT

• Deafness
• Taste and smell disturbance

Urogenital
Management
• Urinary incontinence
• Sexual dysfunction
Pharmacological it is an immunomodulating drug designed Tackling other specific problems
to mimic the effects of the main proteins in Clinical features
myelin; it is given daily by SC injection • Fatigue: trial of amantadine once other causes have
Managing acute relapse:
High-dose steroids (e.g. oral or
• Dimethyl fumarate: an option for treating been excluded
• Spasticity: baclofen and gabapentin are 1st line; other
Complications
adults with active relapsing-remitting
IV methylprednisolone) may be
MS (2 clinically significant relapses in the options include diazepam, dantrolene and tizanidine; Investigations
given for 5 days to shorten the botulinum toxin and cannabis (Sativex spray) can be • Urinary incontinence
previous 2 years) but only if they do not
length of an acute relapse considered if not responding to other treatments • Bowel incontinence
have highly active or rapidly evolving severe
• Neuropathic pain: can be treated with Bloods
Note: steroids shorten the duration relapsing-remitting MS • Depression
of a relapse and do not alter the • Alemtuzumab: an option for treating adults carbamazepine, gabapentin, or using antidepressants • Epilepsy FBC, CRP/ESR, U&Es, LFT, TFT, glucose, HIV serology, calcium and B12
degree of recovery i.e. whether a with active relapsing-remitting MS; it is a such as amitriptyline • Paralysis levels to exclude other causes
patient returns to baseline function monoclonal antibody that binds to CD52, • Bladder dysfunction: anticholinergics may improve
a protein present on the surface of mature urinary frequency if there is no residual volume on Electrophysiology
lymphocytes US scan; intermittent self-catheterisation can be used
Disease-modifying drugs: (a) (b)
• Natalizumab: 2nd-line treatment for MS; it for urine retention Visual, auditory and somatosensory evoked potentials may be
• Beta interferon: shown to is a recombinant monoclonal antibody that prolonged
reduce the relapse rate of MS; it antagonises alpha-4 beta-1-integrin found Non-pharmacological
is given by SC or IM injection; the on the surface of leucocytes, thus inhibiting MRI brain and spinal cord
most common side effect is flu- • Access to multidisciplinary team
their migration across the endothelium
like symptoms including physiotherapy and occupational therapy The investigation of choice for diagnosis and shows plaques
across the blood–brain barrier; it is given
• Glatiramer acetate: licensed • Referral to speech and language therapist for dysarthria particularly in the periventricular area and brainstem (see Fig. 2)
monthly by IV infusion Fig. 2 Radiological appearance of MS
for reducing the frequency of • Mindfulness training and CBT for fatigue; aerobic
• Fingolimod: 2nd-line treatment for MS; it is a Lumbar puncture plaques in (a) T2W axial and (b) sagittal
relapses in ambulatory patients exercise or yoga may also be beneficial
sphingosine 1-phosphate receptor modulator, MRI images
with relapsing-remitting MS • Inform patients of their legal obligation to notify the
and prevents lymphocytes from leaving Shows rise in total protein with increase in immunoglobulin
who have had at least 2 clinical DVLA of their condition
lymph nodes; an oral formulation is available concentration with presence of oligoclonal bands
relapses in the previous 2 years;

204 / Chapter 6 Neurology: Multiple sclerosis Mind Maps for Medicine \ 205
 The key feature is muscle fatigability – muscles become progressively weaker
during periods of activity and slowly improve after periods of rest:
• MG is an autoimmune disease in which • Extraocular muscle weakness: diplopia
antibodies result in a loss of muscle • Proximal muscle weakness: face, neck, limb girdle • Autoantibodies Around 85–90% of patients have antibodies to
Myasthenia gravis (MG) is an acetylcholine receptors (AChRs) • Ptosis (see Fig. 1) acetylcholine receptors; in the remaining patients, about 40% are
acquired autoimmune disorder • In 85% of cases the antibodies bind to the • Bulbar involvement: dysphagia, dysphonia, dysarthria positive for anti-muscle-specific tyrosine kinase antibodies
resulting in insufficient functioning AChRs themselves and in the remaining • CK Normal
acetylcholine receptors. It is cases the antibodies bind to a different • EMG (single fibre) High sensitivity (92–100%)
characterised by weakness, typically muscle membrane target • CT or MRI thorax To exclude thymoma
of the periocular, facial, bulbar and • There are associations between MG and • MG is more common in women (2:1) • Tensilon test IV edrophonium reduces muscle weakness
girdle muscles. thymic hyperplasia (75% of cases) and • It can occur in any age but peaks occur temporarily (rarely used any more due to the risk of cardiac
thymoma (15%) in the 20–30s and 60–80s arrhythmia)

Definition Pathophysiology Epidemiology Fig. 1 Myasthenia gravis illustration showing Investigations

left-sided ptosis

Clinical features

Notes
Myasthenia gravis

Myasthenia gravis

Complications

• Aspiration pneumonia due

to throat muscle weakness
• Acute respiratory
failure during an
exacerbation Management Triggers Associations Myasthenic crisis

• Long-acting anticholinesterase • Emotional stress • Thymomas in 15% • A reversible life-threatening neurological emergency that affects
inhibitors Pyridostigmine is the • Pregnancy • Autoimmune disorders: pernicious 20–30% of myasthenic patients, usually within the first year of illness; it
preferred symptomatic treatment • Menses anaemia, autoimmune thyroid may be the first indication of the disease
• Immunosuppression • Secondary illness disorders, rheumatoid, SLE • Results in weakness of respiratory muscles; facial muscles may be slack,
Prednisolone initially • Thyroid dysfunction • Thymic hyperplasia in 50–70% and face may be expressionless; patient may be unable to support the
• Plasmapheresis and IV • Trauma head, which will fall onto the chest while the patient is seated; jaw is
immunoglobulin Used for • Temperature extremes slack; voice has a nasal quality; body is limp
myasthenic crisis • Hypokalaemia • Often triggered by medications e.g. aminoglycosides, beta blockers
• Thymectomy Important if a • Drugs: aminoglycosides, • Gag reflex is often absent, and such patients are at risk for aspiration
thymoma is present but may be beta blockers, calcium of oral secretions
beneficial even without one channel blockers, quinidine, • Management: monitor forced vital capacity, ventilatory support,
procainamide, chloroquine, plasmapheresis or IV immunoglobulins
lithium, macrolides, tetracycline,
penicillamine, succinylcholine,
magnesium,
ACE inhibitor
• Surgery

206 / Chapter 6 Neurology: Myasthenia gravis Mind Maps for Medicine \ 207
 NF1 NF1 NF2

• NF1 is a dominantly inherited genetic disorder that results Diagnosis is made if at least 2 of the following are Diagnosis requires at least 1 of the following clinical scenarios:
from a germline mutation in the NF1 tumour-suppressor gene, found (in the absence of 1. Bilateral vestibular schwannomas
neurofibromin, which is located on chromosome 17q11.2 alternative diagnoses): 2. A 1st-degree relative with NF2 and
• About 50% of individuals with NF1 have no family history of the 1. ≥6 Café-au-lait spots or • Unilateral vestibular schwannoma or
Neurofibromatosis (NF) refers to
disease and the disease is due to de novo mutations hyperpigmented macules • Any 2 of: meningioma, schwannoma, glioma, neurofibroma,
a group of genetic disorders that
>5mm in diameter in posterior subcapsular lenticular opacities
primarily affect the cell growth of NF2 prepubertal children and 3. Unilateral vestibular schwannoma and
neural tissues. There are two types:
>15mm postpubertal • Any 2 of: meningioma, schwannoma, glioma, neurofibroma,
• Neurofibromatosis type 1 • NF2 is caused by a mutation in the gene encoding for the
(see Fig. 1) posterior subcapsular lenticular opacities
(NF1), also known as von protein merlin or schwannomin on chromosome 22
2. Axillary or inguinal Fig. 1 Café-au-lait macules 4. Multiple meningiomas and
Recklinghausen’s disease • It is also autosomal dominant although around 50% are
freckles • Unilateral vestibular schwannoma or
• Neurofibromatosis type 2 (NF2) de novo with mosaicism in some
3. ≥2 Typical neurofibromas • Any 2 of: schwannoma, glioma, neurofibroma, cataract
(see Fig. 2) or 1 plexiform
neurofibroma
Definition Pathophysiology 4. Optic nerve glioma
5. ≥2 Iris hamartomas
(Lisch nodules): often
only through slit-lamp
Fig. 2 Neurofibromas
examination by an
ophthalmologist (see Fig. 3)
6. Sphenoid dysplasia
or typical long-bone

Neurofibromatosis
abnormalities such as
pseudarthrosis
7. 1st-degree relative
(e.g. mother, father, sister,
brother) with NF1 Fig. 3 Lisch nodules

Management Clinical features/diagnostic criteria

Complications
NF1
Notes
Neurofibromatosis
NF1 • Multidisciplinary team involvement including geneticist, neurologist,
surgeon and physiotherapist, coordinated by a GP
• Mild learning disability
• Comprehensive examination each year in children e.g. detailed skin
• Nerve root compressions caused by
examination, eye tests, and assessment of bone, behaviour, blood
neurofibromas
pressure, physical ability and progress at school
• GI bleeds/obstruction
• Neurofibromas should not be excised unless they show evidence
• MSK complications: bone-cystic
of malignancy or are causing symptoms
lesions, scoliosis, pseudarthrosis
• Other options include chemotherapy or radiation if a tumour has turned
• Hypertension (from renal artery
malignant or cancerous
stenosis)
• Plexiform neurofibromas may be treated with plastic surgery but there is
• Phaeochromocytoma
risk of paralysis especially if the cranial nerves are involved superficially
• Malignancy
• Cranial and spinal neurofibromas are amenable to corrective surgery
• Optic glioma
• Any gliomas or meningiomas should usually be extirpated, partially
• Increased risk of epilepsy
or completely, once intracranial pressure is raised
• Carcinoid syndrome (rare)
• Genetic counselling
NF2
NF2
• Partial/total deafness and tinnitus
• Annual monitoring usually involving hearing tests, an MRI brain scan
• Facial nerve damage
and eye testing
• Visual disturbance
• Hearing aids and management of tinnitus may be required
• Schwannomas
• Surgery and radiotherapy (less common) are options for brain tumours
• Weakness or numbness in the
depending on size
extremities
• Genetic counselling
• Multiple benign brain tumours

208 / Chapter 6 Neurology: Neurofibromatosis Mind Maps for Medicine \ 209

 Motor (see Fig. 1)
• PD (most common cause)
• Drug induced: antipsychotics, • Tremor Worse at rest and usually improves with Blank facial Forward tilt
metoclopramide, phenothiazines e.g. movement; asymmetrical onset; often ‘pill rolling’ of expression to posture
chlorpromazine thumb over fingers (4–6 cycles/sec)
• Progressive supranuclear palsy • Rigidity/↑tone Lead-pipe and cog-wheel rigidity
or Steele–Richardson–Olszewski • Bradykinesia/hypokinesia Slow to initiate Rigidity
syndrome • The 2 major neuropathological movements, expressionless face Slow, monotonous,
Parkinsonism is an umbrella term for the • Multiple system atrophy (previously findings in PD: slurred speech
• Postural instability May cause falls
clinical syndrome involving bradykinesia Shy–Drager syndrome) • Loss of pigmented dopaminergic Reduced
• Gait disorder ↓Arm swing, festinating (shuffling steps Trembling of arm
plus at least one of tremor, rigidity • Wilson’s disease neurones in the pars compacta of difficult to stop with flexed trunk), freezing extremities swinging
and/or postural instability. Parkinson’s
. • Post encephalitis the substantia nigra
disease (PD) is an idiopathic progressive • Dementia pugilistica or chronic • The presence of Lewy bodies and Non-motor Rigidity and tremor
neurodegenerative condition caused by traumatic encephalopathy (secondary Lewy neurites of extremities
degeneration of dopaminergic neurones to chronic head trauma e.g. boxing) • Approximately 60–80% of • Sense of smell reduced and head
in the substantia nigra of the basal • Toxins: carbon monoxide, MPTP, dopaminergic neurones are lost before • Constipation
ganglia. copper the motor signs of PD emerge • Psychosis: complex visual hallucinations and paranoid
ideation
• Frequency/urgency Short, shuffling gait
Definition Causes Pathophysiology • Dribbling of saliva
• Sweating
• Sleep disorders
• Swallowing difficulties
• Depression
• Dementia

Parkinsonism Clinical features Fig. 1 Typical appearance of parkinsonism

Complications Management Parkinson-plus syndromes Investigations

• Infections, most commonly aspiration Conservative A group of neurodegenerative diseases featuring the classical Note: diagnosis is clinical, and investigations mainly focus on
pneumonia features of PD with additional features that distinguish them excluding other causes of the presentation:
• Bed sores • Education and support, notifying DVLA, carer support, from simple idiopathic PD: • CT or MRI brain For patients who do not respond to
• Poor nutrition access to a multidisciplinary team • Progressive supranuclear palsy Impairment of vertical levodopa; MRI can be used to exclude secondary causes of
• Falls gaze (patients may complain of difficulty reading or parkinsonism e.g. tumours
• Contractures Pharmacological (see Notes) descending stairs), early postural instability, symmetrical • PET, SPECT, DaT scan Used to measure basal ganglia
• Bowel and bladder disorders onset, speech and swallowing problems, little tremor dopaminergic function where diagnosis is unclear
• Dopamine receptor agonists
• Acute akinesia • Multiple system atrophy Early autonomic disturbance • Genetic testing e.g. Huntington’s gene; <5% of all PD
• Levodopa
(postural hypotension, impotence/incontinence) and cases are caused by known single-gene mutations
• MAO-B (monoamine oxidase B) inhibitors
cerebellar signs • Olfactory testing
• COMT (catechol-O-methyltransferase) inhibitors
• Lewy body dementia Fluctuating cognition with visual • Caeruloplasmin levels (rule out Wilson’s disease) and
• Amantadine
hallucinations and early dementia syphilis serology (rule out syphilis) For young-onset or
• Antimuscarinics
• Corticobasal degeneration Akinetic rigidity affecting one atypical disease
Deep brain stimulation/surgery limb, cortical sensory loss, apraxia
• Vascular parkinsonism Pyramidal signs (legs), e.g.
• Considered for people with advanced PD who fail to in diabetic/hypertensive patients who fall or have gait
be controlled by medical therapy, are biologically fit, problems
are levodopa responsive and have no mental health
problems

210 / Chapter 6 Neurology: Parkinsonism Mind Maps for Medicine \ 211

 Parkinsonism notes Notes
Parkinsonism

Pharmacological management

Levodopa • Levodopa (l-dopa) should be offered to people in the early stages of PD whose motor symptoms impact on
their quality of life
• It crosses the blood–brain barrier where it is converted to dopamine
• Usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide) to prevent peripheral
metabolism of l-dopa to dopamine
• Effectiveness reduces with time (usually by 2 years)
• Side effects include: dyskinesia, dry mouth, ‘on-off’ effect, drowsiness, anorexia, palpitations, postural
hypotension, psychosis

Dopamine receptor • Dopamine agonists may be used 1st line as a symptomatic treatment for people with early PD
agonists • Can also be used in advanced disease in conjunction with l-dopa to control fluctuations in response
• May be ergot derived or non-ergot derived
• Non-ergot-derived dopamine agonists are preferred (pramipexole and ropinirole) due to fewer adverse effects
• Ergot-derived drugs (e.g. bromocriptine, cabergoline, lisuride) should not be offered as 1st-line treatment for
PD because of the risk of pulmonary, retroperitoneal and cardiac fibrosis; echocardiogram, ESR, creatinine and
CXR should be obtained prior to treatment and patients should be closely monitored
• Side effects include: impulse control disorders, excessive daytime somnolence, hallucinations, postural
hypotension, nasal congestion

MAO-B (monoamine • e.g. Selegiline, rasagiline

oxidase B) inhibitors • Inhibit the breakdown of dopamine secreted by the dopaminergic neurones
• May be used as a symptomatic treatment for people with early PD or in advanced Parkinson’s to reduce motor
fluctuations

COMT (catechol-O- • e.g. Entacapone, tolcapone

methyltransferase) • Used as a 2nd-line treatment for PD
inhibitors • Used as an adjunct to l-dopa as increases half-life of the drug

Amantadine • May be used as a treatment for people with early PD but should not be used 1st line as response rate is low and
tolerance occurs
• Mechanism of action is not entirely understood; it probably increases dopamine release and inhibits its uptake
at dopaminergic synapses
• Side effects include: ataxia, slurred speech, confusion, dizziness and livedo reticularis

Antimuscarinics • e.g. Procyclidine, benzatropine, trihexyphenidyl

• Block cholinergic receptors
• Used more to treat drug-induced parkinsonism rather than idiopathic PD; help with tremor and rigidity
• Side effects include: dry mouth, constipation, urinary retention, blurred vision

Apomorphine • A potent dopamine agonist

• Useful in patients with severe motor complications to decrease ‘off’ periods and dyskinesia
• Two main treatment options: intermittent subcutaneous rescue injections or via a continuous infusion

Beta blockers • Propranolol may be used as an adjunct for the symptomatic treatment of postural tremor in PD

212 / Chapter 6 Neurology: Parkinsonism notes Mind Maps for Medicine \ 213
 • The pathophysiology of polyneuropathy • Metabolic Diabetes mellitus, renal failure, Polyneuropathies can be classified by time course (acute vs chronic),
depends on the underlying cause hypothyroidism, hypoglycaemia underlying pathology (demyelination vs axonal degeneration) or
• It is usually symmetrical and widespread • Vasculitides Polyarteritis nodosa, by affected function (motor vs sensory)
with distal weakness and sensory loss: rheumatoid arthritis, Wegener’s
‘glove and stocking’ (see Fig. 1) granulomatosis Predominantly motor
• Two pathological processes predominate in • Malignancy Paraneoplastic syndrome,
diseases of peripheral nerves: polycythaemia rubra vera • GBS
• Axonal degeneration The most • Inflammatory Guillain–Barré syndrome • Porphyria
common pathology seen in systemic, (GBS), sarcoidosis, chronic inflammatory • Lead poisoning
metabolic, toxic and nutritional causes demyelinating polyneuropathy (CIDP) • Hereditary sensorimotor neuropathy (HSMN): CMT
Peripheral polyneuropathy is an acute • Segmental demyelination Primary • Nutritional ↓Vitamin B1, B12, vitamin E, • CIDP
or chronic, diffuse, often symmetrical, destruction of the myelin sheath folate • Diphtheria
disease and may involve motor, sensory, leaving the axon intact (note: axonal • Inherited Charcot–Marie–Tooth
or autonomic nerves, either alone or Predominantly sensory
degeneration may also be present in Fig. 1 Typical ‘glove and stocking’ pattern (CMT), porphyria, Refsum syndrome,
in combination. demyelinating neuropathies and vice leucodystrophy
of peripheral neuropathy • Diabetes
versa); an example is GBS • Toxins/drugs Alcohol, vincristine, • Alcoholism
nitrofurantoin, phenytoin, metronidazole, • Vitamin B12 deficiency
Definition Pathophysiology
lead, arsenic
• Others Amyloidosis, paraproteinaemias
• Uraemia
• Leprosy
• Amyloidosis
Causes
Classification

Polyneuropathies
Clinical features

Investigations
Sensory symptoms

Management • Urine Dipstick (for glucose, protein), urine ACR and • Numbness, tingling, pins and needles in the hands and feet
Bence Jones proteins • Burning sensations
• Bloods FBC, ESR, vitamin B12, folate, fasting glucose/ • Pain in the extremities
HbA1c, U&Es, LFTs, TFTs, serum electrophoresis, • Sensations of ‘walking on cotton wool’
Treat underlying cause Management of neuropathic pain RF, ANA, anti-ganglioside antibodies, anti-Ro and • Band-like sensations around the wrists or ankles
anti-La antibodies, anti-neuronal antibodies (Hu, Yo) • Unsteadiness on the feet, or stumbling
For example, good diabetes control, treat nutritional Non-pharmacological • Nerve conduction studies Helpful in
deficiencies e.g. vitamin B12/folate, treat underlying • Acupuncture Motor symptoms
differentiating primary demyelination from axonal
malignancy, alcohol cessation, IV immunoglobulins in • Transcutaneous electrical nerve stimulation (TENS) Non-invasive degeneration
GBS (see Ch6: Guillain–Barré syndrome) therapeutic option which uses peripheral nerve electrical stimulation • Weakness or clumsiness of the limbs
• Lumbar puncture Helps to diagnose GBS and CIDP
by means of electrodes placed on the skin surface at known well- • Genetic testing e.g. For CMT • Difficulty walking (including falls and stumbling)
Supportive management • Wasting of affected muscles
tolerated intensities • Nerve biopsy May be required
• Percutaneous electrical nerve stimulation (PENS) Uses needle-like • Reduced/absent reflexes
• Physio/occupational therapy involvement for mobility,
electrodes (similar to those used in acupuncture) located in soft tissues • Respiratory difficulty
muscle strengthening and home adaptation
• Walking aids, e.g. a stick or Zimmer frame, for support or muscles at the corresponding dermatomes for that local pathology
Autonomic neuropathy
• Foot care and shoe choice are very important in
sensory neuropathies to minimise trauma Pharmacological • Postural hypotension
• Neuropathic-specific analgesics such as duloxetine, amitriptyline, • Erectile dysfunction
gabapentin, or pregabalin • Anhidrosis
• Other options include tramadol (short term) and capsaicin cream (for • Constipation or diarrhoea
people with localised neuropathic pain who wish to avoid or cannot • Urinary retention
tolerate oral treatments) • Horner syndrome
• Cardiac arrhythmias
• Holmes–Adie pupil

214 / Chapter 6 Neurology: Polyneuropathies Mind Maps for Medicine \ 215

 Lifestyle factors Other factors Carotid territory symptoms

• Smoking • Increasing age • Amaurosis fugax

• Alcohol misuse and drug abuse e.g. cocaine, • Gender: men, ↑risk at younger • Aphasia
methamphetamine age; women, ↑stroke risk linked • Hemiparesis
• The cause is usually embolic; it may • Physical inactivity to oral contraceptives, migraine • Hemisensory loss
Transient ischaemic attack, sometimes be thrombotic, and occasionally • Poor diet with aura, the immediate • Hemianopic visual loss
referred to as ‘mini stroke’, refers to haemorrhagic postpartum period and
transient (<24h) neurological dysfunction • The most common source of emboli is the Established cardiovascular disease pre-eclampsia Vertebrobasilar territory symptoms
caused by focal brain, spinal cord or carotids, usually at the bifurcation; they • Peripheral vascular disease
retinal ischaemia, without evidence may originate in the heart (particularly • Hypertension • Hyperlipidaemia • Diplopia
of acute infarction. in AF); the vertebrobasilar arteries may • AF (causes more than 20% of ischaemic strokes) • Diabetes mellitus • Vertigo
also be a source • Ischaemic heart disease • Sickle cell disease • Vomiting
• Infective endocarditis • Antiphospholipid syndrome and • Choking and dysarthria
• Valvular heart disease other hypercoagulable disorders • Ataxia
Definition Causes •
•
Congestive heart failure
Congenital or structural heart disease
• Chronic kidney disease •
•
Hemisensory loss
Hemianopic or bilateral visual loss
• Obstructive sleep apnoea
• Tetraparesis
• Loss of consciousness

Risk factors
Clinical features

Transient ischaemic attack

Management Investigations Differential diagnosis ABCD2 score

Initial Long-term • Bloods FBC, ESR/CRP, U&Es, glucose, LFTs, TFTs, • Stroke Estimates stroke risk after suspected TIA but
cholesterol, clotting and antiphospholipid • Hypoglycaemia should not be used any more for deciding
• Aspirin 300mg Unless • Control risk factors e.g. HTN and diabetes, antibodies • Migraine with aura management:
contraindicated should be given obesity, smoking cessation • ECG/24-h ECG Look for arrhythmias • Focal epilepsy • Age ≥60 years (1 point)
immediately; if the patient is • Antiplatelet long term Clopidogrel e.g. AF/paroxysmal AF • Intracranial lesion e.g. tumour or haemorrhage • Blood pressure ≥140/90mmHg (1 point)
already taking low-dose aspirin is recommended 1st line 75mg OD; • CT brain To primarily rule out haemorrhage • Hyperventilation • Clinical features Unilateral weakness
regularly it should be continued aspirin + dipyridamole should be given to • MRI brain To determine the region of ischaemia, • Retinal or vitreous haemorrhage (2 points), speech disturbance without
on the current dose until reviewed patients who cannot tolerate clopidogrel rule out haemorrhage or other pathologies • Labyrinth disorder weakness (1 point)
by a specialist • Anticoagulate In AF (see Ch1: Atrial fibrillation) • Carotid imaging Carotid Doppler and duplex • Malignant hypertension • Duration of symptoms ≥60 min (2 points),
• Specialist assessment by • Long-term statin e.g. Atorvastatin US to look for atheroma and stenosis; MRI or CT 10–59 min (1 point)
stroke physician Within 20–80mg daily angiography may be required • Diabetes Presence of diabetes (1 point)
24h if the patient has had a • Carotid endarterectomy Consider if carotid • Transoesophageal/transthoracic ECHO To rule (low risk: 1–3, moderate risk: 4–5,
suspected TIA <7 days ago and stenosis >70% according to ECST criteria or out transmural thrombus or valvular heart disease high risk: 6–7)
within 7 days if they have had > 50% according to NASCET criteria
suspected TIA >7 days ago • Driving Must not drive for 1 month following a
TIA; if multiple TIAs over a short period, require
3 months free from further attacks before
resuming driving, and DVLA should be notified

216 / Chapter 6 Neurology: Transient ischaemic attack Mind Maps for Medicine \ 217
 Ischaemic stroke (85%) Rarer causes Cerebral hemisphere infarcts (approx. 50%) Brainstem infarcts (approx. 25%)

Occurs when large arteries (such as the • Cerebral venous Most commonly due to infarction of the internal capsule due to Cause complex patterns of dysfunction and depend on the site
extracranial carotid or vertebral arteries), thrombosis: more occlusion of a branch of the middle cerebral artery: involved:
intracranial arteries or small penetrating common in patients in pro- • Contralateral hemiplegia • Lateral medullary syndrome (most common form)
arteries (lacunar) are occluded by: thrombotic state e.g. related • Contralateral hemisensory loss Caused by posterior cerebellar artery occlusion; presents
• Thrombus due to atherosclerosis or to pregnancy, infection, • Homonymous hemianopia with sudden vomiting and vertigo, ipsilateral Horner
• Embolus of fatty material from an dehydration or malignancy • Dysphasia: if the site of the lesion is in the left (usually the syndrome, facial numbness, cerebellar signs and palatal
atherosclerotic plaque or a clot in a • Carotid artery dissection: dominant cerebral hemisphere) then language functions will be paralysis with diminished gag reflex; there may be pain and
larger artery or the heart (often due more common in younger affected loss of temperature sensation on the contralateral side
to AF or atherosclerosis of the carotid people and may be • Upper motor neurone signs, including facial weakness • Coma Due to involvement of reticular activating system
A clinical syndrome characterised arteries) preceded by neck trauma • In right-sided cerebral infarcts there is likely to be neglect of • The locked-in syndrome Caused by upper brainstem
by sudden onset of rapidly • Genetic conditions such as the contralateral limbs, constructional or dressing apraxia, and infarction; all voluntary muscles are paralysed except those
developing focal or global Haemorrhagic stroke (15%) Fabry’s disease and CADASIL topographical agnosia that control eye movement
neurological disturbance which (cerebral autosomal • Pseudobulbar palsy Caused by lower brainstem infarction
lasts more than 24h or leads to
• Intracerebral haemorrhage (main dominant arteriopathy with Lacunar infarcts (approx. 25%)
cause is hypertension) subcortical infarcts and Same as TIA (see Ch6:
death.
• Subarachnoid haemorrhage Caused by small infarcts that produce localised deficits:
leucoencephalopathy) Transient ischaemic attack)
• Pure sensory, pure motor or mixed motor and sensory deficit
• Sudden-onset unilateral cerebellar ataxia and sudden dysarthria
Definition with a clumsy hand are typically caused by single lacunar infarct Same as TIA (see Ch6: Transient
Causes Risk factors ischaemic attack)

Clinical features Investigations

Stroke

Complications Assessment tools

Management

Acute stroke management Thrombolysis

Acute Long-term FAST (for general public use)
• Arrange urgent CT head (see Fig. 1) Alteplase should only be given if administered <4.5h from
or MRI scan onset of stroke symptoms and haemorrhage has been • Haemorrhagic transformation of • Mobility problems: hemiparesis or • Facial weakness Can they smile? Has their mouth
• Blood glucose, hydration, oxygen definitively excluded (see Notes: Thrombolysis contraindications). ischaemic stroke hemiplegia, ataxia, falls, spasticity and or eye drooped?
saturations and temperature should • Cerebral oedema contractures • Arm weakness Can they raise both arms?
be maintained within normal limits; Thrombectomy • Seizures • Sensory deficits • Speech problems Can they speak clearly and can they
(hypertension and labile BP are common • Venous thromboembolism: • Urinary and faecal incontinence understand what you’re saying?
on presentation and should not be acutely Mechanical thrombectomy is a relatively new treatment option pulmonary embolism • Long-term pain (neuropathic or • Time It is time to call 999 immediately if you see any
corrected) for patients with an acute ischaemic stroke. All decisions about • Cardiac complications: MI, heart musculoskeletal) of these symptoms
• Give aspirin 300mg orally or rectally (if thrombectomy should take into account a patient’s overall failure, AF and arrhythmias are • Fatigue
clinical status. common • Dysphagia, poor oral hygiene, dehydration Rosier score (for medical professionals)
unable to swallow) ASAP if a haemorrhagic
stroke has been excluded • Infection: including aspiration and malnutrition
Neurosurgery pneumonia, UTI and cellulitis from • Sexual dysfunction Exclude hypoglycaemia first, then assess the following
• Admit to stroke unit (a stroke is likely if >0):
• NBM if swallowing deemed unsafe with Fig. 1 Axial non-contrast CT head infected pressure sores • Pressure sores
This is for patients with haemorrhagic stroke. • Visual problems: altered acuity, hemianopia, • Loss of consciousness or syncope (–1 point)
SALT assessment and support with feeding showing right middle cerebral artery
diplopia, nystagmus and blurred vision • Seizure activity (–1 point)
• Prevention of DVT with early mobilisation infarct (darker area) Long-term stroke management (see Notes)
• Cognitive problems • New, acute onset of:
and TED stockings • Weakness Asymmetric facial weakness (+1 point),
• Difficulties with activities of daily living (ADL)
• Anxiety and depression asymmetric arm weakness (+1 point), asymmetric
• Communication problems: dysphasia and leg weakness (+1 point)
dysarthria • Speech disturbance (+1 point)
• Visual field defect (+1 point)

218 / Chapter 6 Neurology: Stroke Mind Maps for Medicine \ 219

 Stroke notes Notes
Acute respiratory distress syndrome
Stroke

Thrombolysis contraindications Management of long-term complications Barthel index for ADL

• Swallowing or speech difficulty Assessment and ongoing management Assesses functional independence in stroke patients:
Absolute Relative by speech and language therapist
• Nutrition and hydration Refer to a dietitian if oral intake of nutrition and Bowels 0 Incontinent
• Previous intracranial haemorrhage • Concurrent fluids is inadequate, or if food or fluid consistency needs to be modified
• Incontinence Ongoing incontinence should be followed up by community 5 Occasional accident
• Seizure at onset of stroke anticoagulation
• Intracranial neoplasm (INR >1.7) continence services 10 Continent
• Suspected subarachnoid • Haemorrhagic diathesis • Cognitive dysfunction Consider screening for cognitive impairment and
referring for a neuropsychological assessment if this is suspected Bladder 0 Incontinent
haemorrhage • Active diabetic
• Stroke or traumatic brain injury in haemorrhagic • Depression and anxiety Screen all patients for this and treat appropriately 5 Occasional accident
preceding 3 months retinopathy • Mouth care Advise people with stroke (particularly those who are tube fed
or have swallowing problems) and their carers to carry out mouth care at 10 Continent
• Lumbar puncture in preceding 7 days • Suspected intracardiac
• GI haemorrhage in preceding 3 weeks thrombus least 3 times a day Grooming 0 Needs help with personal care
• Active bleeding • Major surgery/trauma in • Visual problems Patients should be referred to an orthoptist or
ophthalmologist specialising in stroke if there are any visual complications 5 Independent (face, hair, teeth, shaving)
• Pregnancy the preceding 2 weeks
• Oesophageal varices • Long-term pain Treat people with central post-stroke pain with
Toilet use 0 Dependent
• Uncontrolled hypertension amitriptyline, gabapentin or pregabalin; people with ongoing
>200/120mmHg musculoskeletal pain should initially be treated with simple analgesic drugs 5 Needs some help (can do some things alone)
such as paracetamol and NSAIDs
10 Independent (on and off dressing and wiping)
• Sexual dysfunction Patients should be screened for sexual dysfunction
• Spasticity and contractures Spasticity and development of contractures Feeding 0 Unable
Long-term management of stroke are common following stroke and can lead to discomfort and pain; splinting
5 Needs help with cutting, spreading butter etc.
and physiotherapy may be useful
Secondary prevention • End of life Those with stroke who are nearing the end of life should have 10 Independent
access to the specialist palliative care team when needed
• Control risk factors e.g. HTN and diabetes, obesity, smoking cessation Transfer 0 Unable, no sitting balance
• Antiplatelet long term Clopidogrel is recommended 1st line 75mg OD Driving (bed to chair
5 Major help needed (physical, 1–2 people), can sit
long term; aspirin + dipyridamole should be given to patients who cannot and back)
tolerate clopidogrel • Patients who are group 1 vehicle drivers cannot drive for 1 month after a 10 Minor help needed (verbal or physical)
• Anticoagulate in AF (see Ch1: Atrial fibrillation) stroke provided there are no residual deficits; they do not need to inform the
DVLA 15 Independent
• Long-term statin e.g. Atorvastatin 20–80mg daily
• Carotid endarterectomy Should be considered if carotid stenosis >70% • Patients who drive large goods vehicles or passenger-carrying vehicles must Mobility 0 Immobile
according to ECST criteria or >50% according to NASCET criteria inform the DVLA (on level
5 Wheelchair independent, including corners
surfaces)
Stroke neurorehabilitation 10 Walks with help of 1 person
• Optimal treatment is on a stroke rehabilitation unit that provides 15 Independent
multidisciplinary service
Dressing 0 Dependent
• Early physiotherapy is particularly useful in the first few months in
reducing spasticity, relieving contractures and helping with walking aids 5 Needs some help but can do some things alone
• Occupational therapists play a vital role in assessing the requirement for
and arranging various aids and home modification 10 Independent (including buttons, zips, laces)
Stairs 0 Unable
5 Needs help (verbal, physical, carrying aid)
10 Independent
Bathing/ 0 Dependent
showering
5 Independent

Interpretation:
80–100 Independent
60–79 Minimally dependent
40–59 Partially dependent
20–39 Very dependent
<20 Totally dependent

220 / Chapter 6 Neurology: Stroke notes Mind Maps for Medicine \ 221
 Anterior
communicating The Hunt and Hess Classification Notes
artery Subarachnoid haemorrhage
of Subarachnoid Haemorrhage
Anterior
Classifies severity of subarachnoid
cerebral • Hypertension haemorrhage to predict mortality:
• Spontaneous rupture of berry • Smoking • Grade 1 Asymptomatic, mild
artery
aneurysms (85%): conditions Middle • Cocaine use headache, slight nuchal rigidity
Subarachnoid haemorrhage (SAH) associated with berry aneurysms 40% cerebral artery
• Excessive alcohol • Grade 2 Moderate–severe
is bleeding into the subarachnoid include adult polycystic kidney • Linked genetic disorders include headache, nuchal rigidity, no
space and is a medical emergency. It disease, Ehlers–Danlos syndrome Internal autosomal dominant adult neurological deficit other than
and coarctation of the aorta 20%
is usually as a result of bleeding from carotid artery 34% polycystic disease, Ehlers– cranial nerve palsy
an aneurysm in the circle of Willis • AV malformations Danlos syndrome type IV and • Grade 3 Drowsiness/confusion,
4%
(see Fig. 1). • Trauma Posterior neurofibromatosis type 1, mild neurological deficit
• Tumours communicating Marfan syndrome • Grade 4 Stupor, moderate–severe
artery
Posterior • 1st-degree relatives have a 3–7× hemiparesis
Definition cerebral artery relative risk compared with the • Grade 5 Coma, decerebrate
Causes Basilar artery general population posturing

Fig. 1 The main cerebral arteries showing the circle of

Willis and the most common sites for berry aneurysms Risk factors Classification

Subarachnoid haemorrhage

Complications Management Investigations Clinical features

• Re-bleeding (in 30%) • Bed rest and supportive • CT head scan The investigation of choice and should be done • Headache: ‘sudden onset, ‘worst ever’,
• Obstructive hydrocephalus measures with cautious control as soon as possible; detects SAH in 95% of cases with scanning ‘thunderclap’
(due to blood in ventricles) of hypertension within 24h of haemorrhage; the sensitivity decreases with time • Vomiting
• Cerebral ischaemia • Nimodipine, e.g. 60mg 4-hourly (see Fig. 2) • Neck stiffness and positive Kernig’s sign
• Death orally or by IV infusion, has been • Bloods FBC (check platelet count prior to lumbar puncture), • Photophobia
shown to reduce the severity of U&Es, clotting screen • Drowsiness
neurological deficits but does • ECG Peaked P and T waves, short PR interval, prolonged QT • Confusion
not reduce re-bleeding interval, tall U waves • Unilateral eye pain
• Neurosurgical opinion: no clear • Lumbar puncture If • Loss of consciousness
evidence over early surgical the CT head is normal. A • Seizure
intervention against delayed lumbar puncture must not • Coma
intervention be performed if there are • Reactive hypertension
features of raised intracranial • Focal neurological signs
pressure; if performed within • Fundoscopy: subhyaloid
6–12h then CSF is uniformly haemorrhages, with or without
bloodstained; if performed papilloedema
between 12h and 2 weeks
after initial headache then
the CSF is xanthochromic
• CT/MR angiography
Usually performed to
Fig. 2 Left temporal lobe subarachnoid
establish the source of
haemorrhage on CT scan
bleeding in all patients
potentially fit for surgery

222 / Chapter 6 Neurology: Subarachnoid haemorrhage Mind Maps for Medicine \ 223
 An acute SDH is usually caused by: • History of trauma: often minor and
• Tearing of bridging veins from the cortex the latent interval between injury and
to one of the draining venous sinuses – symptoms may be weeks or months
typically occurring when bridging veins • Acute This phase begins less than 3 days • Fluctuating conscious level:
are sheared during rapid acceleration– after initial injury • Any factor that stretches the bridging • There may be a history of gradual-
deceleration of the head • Subacute This phase begins 3–7 days after the veins: onset of headaches, memory loss,
• Bleeding from a damaged cortical artery initial injury • Cerebral atrophy, e.g. elderly personality change, confusion and
• Blunt head trauma is the usual mechanism • Chronic This phase begins 2–3 weeks after the • Low CSF pressure after shunting, e.g. drowsiness
of injury but spontaneous SDH can arise initial injury for long-standing hydrocephalus or • Symptoms vary from day to day
as a consequence of clotting disorder, • Simple SDH There is no associated parenchymal a fistula with intervening lucid periods
A subdural haematoma (SDH) is a arteriovenous malformations/aneurysms or injury • Alcoholism • Focal neurological signs: often,
collection of blood deep in the dural other conditions • Complicated SDH There is associated underlying • Coagulation disorder or hemiparesis
layer of the meninges. • In the subacute phase, the collection of parenchymal injury, such as contusion anticoagulation therapy e.g. warfarin • Aphasia: if the lesion is on the left side
clotted blood liquefies; in the chronic phase
it becomes a collection of serous fluid in the
subdural space
Definition Classification Risk factors Clinical features
Pathophysiology

Subdural haematoma

Investigations

Complications Management Bloods

• FBC, U&Es and LFTs may reveal alternative

causes of impaired consciousness;
• Death due to cerebellar herniation • ABC approach of resuscitation thrombocytopenia may indicate a bleeding
• Raised intracranial pressure • Intubation and assisted ventilation may be needed depending on diathesis
• Cerebral oedema level of consciousness • Coagulation screen: to screen for coagulopathy
• Recurrent haematoma formation • In cases of severe trauma, the cervical spine should be immobilised • Group and save/cross-match if SDH seems likely,
during recovery and the trauma team should be alerted in anticipation of operative intervention
• Seizures • If suspected or confirmed by investigation, refer urgently to the
• Wound infection, subdural neurosurgical team Imaging
empyema, meningitis • Treat any coagulopathy, e.g. vitamin K, fresh frozen plasma, for Fig. 1 Left acute subdural
• Permanent neurological or reversing the effects of warfarin • CT head 1st-line investigation; shows a
haematoma on CT scan
cognitive deficit due to pressure • Measures to decrease raised intracranial pressure include IV crescentic collection, not limited by suture
effects on the brain mannitol or hypertonic saline lines; acute subdural haematomas will appear
• Coma/persistent vegetative state • Burr holes may be considered if there is rapid deterioration hyperdense (bright) in comparison with the
• For a small asymptomatic acute SDH, this can be managed by brain (see Fig. 1); chronic SDHs are hypodense
observation, serial examinations and serial CT scanning (dark) compared with the substance of the brain
• Surgery is needed if there are focal signs, deterioration, a large (see Fig. 2)
haematoma, raised intracranial pressure or midline shift • Skull X-ray May reveal skull fracture
• SDH is treated by emergency craniotomy and clot evacuation • MRI head Can be used to detect SDH
• The use of a drain can reduce the risk of recurrence

Fig. 2 Left chronic subdural

haematoma on CT scan
224 / Chapter 6 Neurology: Subdural haematoma Mind Maps for Medicine \ 225
 Chapter 7
07
Rheumatology

Ankylosing spondylitis...........................................................................................................................................228
Fibromyalgia.................................................................................................................................................................230
Giant cell arteritis.......................................................................................................................................................232
Gout...................................................................................................................................................................................234
Osteoarthritis................................................................................................................................................................236
Osteoporosis.................................................................................................................................................................238
Paget’s disease............................................................................................................................................................240
Polymyalgia rheumatica........................................................................................................................................242
Polymyositis and dermatomyositis.................................................................................................................244
Psoriatic arthritis.........................................................................................................................................................246
Reactive arthritis.........................................................................................................................................................248
Rheumatoid arthritis................................................................................................................................................250
Scleroderma..................................................................................................................................................................254
Septic arthritis..............................................................................................................................................................256
Sjögren syndrome.....................................................................................................................................................258
Systemic lupus erythematosus.........................................................................................................................260
Vitamin D deficiency...............................................................................................................................................264

Mind Maps for Medicine \ 227

 • Both genetic and (a) (b) (c) Articular • Atrioventricular node block
environmental factors • Achilles tendonitis
contribute to AS Inflammation • Back pain and stiffness Typically radiating • Amyloidosis (rare and late complication)
• HLA-B27 is the most common from the SI joints to the hips/buttocks
Fusion The modified Schober’s test examines the degree of flexion
predisposing gene in AS • Reduced motion In the lumbar and
• The disease is first cervical spine of the spine (see Fig. 2):
Ankylosing spondylitis (AS) is a chronic characterised by inflammation 1. An inferior mark at the level of the posterior superior
• Loss of lumbar lordosis
seronegative spondyloarthropathy of the SI joints; in the later Fig. 1 (a) Normal spine, (b) early AS, (c) advanced AS iliac spine is drawn and a 10-cm segment above this is
• Reduced chest expansion
which primarily involves the sacroiliac stages the annulus fibrosus • HLA-B27 gene: 90% of AS patients marked
• Thoracic kyphosis and neck
(SI) joints and the spine. Other clinical starts to calcify, creating a bony bridge between the vertebral bodies (syndesmophytes) are carriers 2. The increase in distance on maximum forward flexion
hyperextension ‘Question mark posture’
features include peripheral arthritis, • These may then fuse with the vertebral body above causing ankyloses (see Fig. 1). • Men (3:1) with locked knees is measured
• Peripheral synovitis Typically
enthesitis and extra-articular organ • Age 17–35 years 3. The measured distance should increase from 10cm to at
asymmetrical oligoarthritis (most
involvement. • Northern European least 13.5–15cm in a healthy adult
commonly affects the hip and knee)
Pathophysiology
Extra-articular (The ‘A’ factors)
Definition Risk factors 10 cm 15 cm
• Atlanto-axial subluxation
• Anterior uveitis
• Apical lung fibrosis
• Aortic valve incompetence
Fig. 2 Modified Schober’s test

Clinical features

Ankylosing spondylitis

Investigations Notes
Ankylosing spondylitis

Imaging
Management
• X-rays:
• Early stages: may be normal or
Conservative
there may be bony erosions,
• Patient education widening of SI joints and
• Exercise squaring of vertebral bodies
• Physiotherapy with shiny corners (Romanus
• Hydrotherapy and swimming are excellent activities to maintain mobility and fitness lesions)
• Later stages: ossification of
Pharmacological the longitudinal ligaments of
the spine (syndesmophytes)
• NSAIDs 1st line for pain and stiffness giving the spine a bamboo
• Other analgesics When NSAIDs are insufficient e.g. paracetamol and codeine appearance (see Fig. 3)
Fig. 3 X-ray of a ‘bamboo spine’ in AS
• Steroids Local corticosteroid injections are useful for symptomatic sacroiliitis, peripheral • MRI: of the sacroiliac joints is
enthesitis and arthritis; oral corticosteroids can be used for short-term relief of symptoms more sensitive than either plain X-ray or CT scan in demonstrating
• Anti-TNF-alpha therapy e.g. Etanercept and adalimumab are effective in AS that is poorly sacroiliitis
controlled with NSAIDs • Ultrasound: Can help in diagnosing enthesitis
• Bisphosphonates Often used to treat osteoporosis and reduce the risk of fractures in AS
Bloods
Surgical
• FBC Usually normal
• Vertebral osteotomy May be performed to correct spinal deformities, but may cause • ESR/CRP ↑ In active disease
significant neurological complications. • ANA/RF Negative
• Joint replacement Patients may need total hip replacement and, occasionally, total • ALP Often elevated
shoulder replacement. • HLA-B27 +ve in 90% of patients but has little role in diagnosis. It may
indicate AS predisposition in appropriate clinical context
228 / Chapter 7 Rheumatology: Ankylosing spondylitis Mind Maps for Medicine \ 229
 • Pain at multiple sites
• Fatigue
Fibromyalgia is a syndrome of chronic • Insomnia
pain and the presence of hyperalgesic • The cause of fibromyalgia is poorly • The nociceptive system has • Morning stiffness
points at specific anatomical sites, understood but abnormal central links with the stress-regulating, • Paraesthesia
as well as a range of other physical and peripheral pain processing immune and sleep systems which • Feeling of swollen joints (with no objective swelling)
is thought to be responsible may explain some of the clinical • Female: 10× more likely to be affected
and psychological symptoms with no • Problems with cognition (e.g. memory disturbance,
for reduced pain threshold, features • Age: common in individuals aged 20–50 years but it can occur in
identifiable organic cause. It is not a difficulty with word finding)
hyperalgesia (amplification • Genetic and environmental factors any age
diagnosis of exclusion and can occur in • Headaches
of pain) and allodynia (pain may play a role in fibromyalgia as • Physical trauma: e.g. whiplash-type injuries to the neck and trunk
patients with other conditions such as • Light-headedness or dizziness
produced by non-noxious stimuli) it is more common in the relatives • Stress, anxiety and depression
inflammatory arthritis and osteoarthritis. • Fluctuations in weight
of affected patients • Life events: failing to complete education, low income, divorce
• Viral infections: may occur as a post-viral syndrome • Anxiety and depression
(Symptoms are generally reported as worse in cold, humid
weather and under times of stress)
Definition Pathophysiology Risk factors

Clinical features

Notes
Fibromyalgia
Fibromyalgia

Investigations

All investigations including blood tests and imaging are normal. The American
College of Rheumatology criteria for the classification of fibromyalgia include:
Management • Widespread pain: above and below the waist as well as the axial skeleton
for at least 3 months
• Presence of 11/18 tender points shown in Fig. 1
Non-pharmacological Pharmacological Note: The thumb should be used for digital palpation of tender points; the pressure
applied should be just enough to blanch the examiner’s thumbnail. In the absence
• Explanation and education Analgesia: of fibromyalgia, the palpation would not be enough to cause pain.
• Exercise programmes including • Paracetamol, weak opioids and tramadol can be used for the
aerobic exercise and strength management of pain
training • Pregabalin and gabapentin have a small benefit in reducing
• Cognitive behavioural therapy Occiput Low cervical
pain and insomnia Trapezius
may help some patients with Second rib
• Corticosteroids and strong opioids are not recommended Supraspinatus
fibromyalgia
Lateral
• Therapies including relaxation,
rehabilitation, physiotherapy and
Antidepressants: epicondyle
Gluteal
psychological support may help • Can help to reduce pain and improve function
• Tricyclics e.g. amitriptyline have been found to be the best for Greater
some patients trochanter
pain
• SNRIs, e.g. venlafaxine and duloxetine, may be useful in treating Knee
pain and low mood
• SSRIs, e.g. fluoxetine, for low mood

Fig. 1 Common distribution of hyperalgesic tender points in fibromyalgia

230 / Chapter 7 Rheumatology: Fibromyalgia Mind Maps for Medicine \ 231

 Symptoms Signs

• Headache (85%) Usually one-sided (often in the • Scalp tenderness/tenderness over the temporal
• GCA is an autoimmune disorder where exposure temporal or occipital region), commonly worse at artery
to an unknown environmental trigger causes night and tender to touch • Decreased temporal artery pulse
Giant cell arteritis (GCA) is a breakdown of immune tolerance, resulting in an • Polymyalgia rheumatica (PMR): • Visual symptoms Amaurosis fugax (sudden • Swollen temporal artery (see Fig. 1).
systemic immune-mediated autoimmune reaction against the arterial wall 50% of patients with GCA have PMR transient loss of vision in one eye), blurred vision, • Carotid bruits (may be heard on auscultation)
vasculitis affecting medium- and • GCA mainly affects the extracranial branches of the • Age: almost exclusively in patients diplopia, partial or complete loss of vision • Muscle/joint tenderness (if PMR also present)
large-sized arteries, particularly the carotid artery, specifically the temporal artery >50 years • Jaw and tongue claudication (65%) • Abdominal bruits or abnormal pulsatile aneurysm
carotid artery and its extracranial • The histopathological hallmark of GCA is the • Females: 3× more common than males • Systemic features of PMR Muscle ache, fever, swelling
branches. GCA can cause sudden predominance of mononuclear infiltrates of • Caucasians fatigue, weight loss • Pale optic disc Fig. 1 Swollen temporal artery in
vision loss and is therefore a medical granulomas consisting of mainly multinucleated a patient with GCA
emergency. giant cells
Risk factors Clinical features
Definition Pathophysiology

Notes
Giant cell arteritis

Giant cell arteritis

Diagnostic ACR criteria (NEAT)

Management 1. New-onset headache (localised pain in head)

Complications 2. Elevated ESR (≥50mm/h)
3. Abnormal artery biopsy: mononuclear cell infiltration or
Urgent ophthalmology review and start granulomatous lesions (usually with multinucleated giant cells)
• Permanent loss of vision 4. Age of onset ≥50 years
high-dose steroids immediately
(partial or total) 5. Temporal artery abnormality (tenderness on palpation or
• Aneurysms, dissections and ↓pulsation)
stenotic lesions of the aorta
and its major branches (3/5 → high risk of GCA)
• Central nervous system
disease e.g. seizures, CVA
• Steroid-related complications
GCA with no visual Complicated GCA
symptoms with visual symptoms

Prednisolone
Prednisolone
60–100mg oral or IV Investigations
40–60mg oral
methylprednisolone

• Bloods ESR elevated (typically >50mm/h

but <30mm/h in 10% of patients); CRP may
be elevated; FBC: may show normocytic
normochromic anaemia, thrombocytosis may
Tapering regimen e.g. 40–60mg prednisolone for 4 weeks, then reduce be present
by 10mg every 2 weeks to 20mg, then by 2.5mg every 2–4 weeks • Colour duplex US Shown to be relatively
to 10mg, then 1mg every 1–2 months accurate at diagnosing GCA
Bone protection Bisphosphonate and calcium/vitamin D • Temporal artery biopsy May show a typical
supplementation strongly recommended while on steroids appearance of intermittent inflammation (‘skip
to prevent osteoporosis lesions’); may be negative in approximately
20–30% of cases

232 / Chapter 7 Rheumatology: Giant cell arteritis Mind Maps for Medicine \ 233
 • There is a strong association between gouty • Male gender: 4:1 Acute gout
arthropathy and hyperuricaemia which is often • Diet: meat, seafood, oily fish and yeast products
Gout is a disorder of purine asymptomatic for years before the initial attack • Alcohol • Presents as an excruciatingly painful red joint that
metabolism characterised by a • The build-up of urate crystals can be caused • Drugs: e.g. diuretics, chemotherapy is tender and warm to touch (see Fig. 1)
raised uric acid level in the blood by decreased renal excretion e.g. chronic • Obesity • Most commonly affects the metatarsophalangeal
(hyperuricaemia) and the kidney disease, diuretics and overproduction • Hypertension joint (podagra) in about 70% of cases
deposition of urate crystals in joints of uric acid e.g. myeloproliferative disorders or • Coronary heart disease • Other common sites include small joints of the
and other tissues, such as soft overconsumption of purine-rich food that are • Diabetes mellitus foot (mid-tarsal), hands, ankle, knee and elbow
connective tissues or the urinary metabolised to urate • Chronic kidney disease
tract. Gouty arthritis is arthritis due to • When these crystals deposit in the synovial fluid • High triglycerides Chronic gout
of joints they cause gouty arthritis • Heart failure Fig. 1 Gout of the big toe
urate crystals in joints.
• Psoriasis • Usually affects more than one joint (polyarthritis)
• Lesch–Nyhan syndrome • Tophi (subcutaneous deposition of uric acid
Definition Pathophysiology crystals) (see Fig. 2)
• Fever and malaise may be present (uncommon)
Risk factors • Uric acid kidney stones may also develop

Clinical features

Gout Fig. 2 Chronic tophaceous gout

Complications
• Chronic urate nephropathy
• Severe degenerative arthritis
• Secondary infections
• Recurrent painful episodes
• Carpal tunnel syndrome (rare)
• Nerve or spinal cord impingement

Pseudogout Investigations

• The deposition of calcium pyrophosphate Bloods

crystals in the joint space
Management • Presents in a similar way to gout but usually • Serum urate is often ↑ but may fall during an acute attack; useful for
affects larger joints e.g. knee, wrist and ankle monitoring response to treatment
• X-ray may show chondrocalcinosis • Fasting glucose and lipids to rule out hyperglycaemia and
(calcification of cartilage) and OA changes hyperlipidaemia, as gout is commonly associated with
• Aspiration of the joint and synovial fluid metabolic syndrome
Acute gout Prevention analysis → positively birefringent
rhomboid-shaped crystals under X-ray
• NSAIDs1st-line treatment for acute gout in the absence of contraindications • Lifestyle changes Lose weight, ↓excessive consumption of food rich in polarised light microscopy
• Colchicine Used if NSAIDs are contraindicated, not tolerated or have purines e.g. meat and seafood, ↓alcohol consumption, regular exercise, • Acute attacks are as per gout, but allopurinol • Early: soft tissue swelling
previously been ineffective smoking cessation, stop offending drugs if possible has no role in prevention of pseudogout • Later: punched-out erosions, areas of sclerosis, tophi
• Corticosteroids If NSAIDs and colchicine are contraindicated e.g. in renal • Allopurinol 1st line; recommended for recurrent attacks (≥2 attacks in • US/CT/MRI: can identify urate deposition, structural joint damage and
impairment; a course of oral corticosteroids or intra-articular steroids can 12 months), tophi, renal disease, uric acid renal stones and prophylaxis if on joint inflammation in gout
be given cytotoxics or diuretics; start 1–2 weeks after acute gout attack has resolved;
• Canakinumab A recombinant monoclonal antibody licensed for use in co-prescribe NSAID or low-dose colchicine for at least 1 month to prevent Joint aspiration and synovial fluid analysis
patients whose condition has not responded adequately to treatment with gout attack; avoid stopping allopurinol in subsequent gout attacks
• Used to rule out septic arthritis as a differential diagnosis
NSAIDs or colchicine, or who are intolerant of them • Febuxostat 2nd-line therapy if allopurinol is contraindicated/not tolerated
• Provides definitive diagnosis of gout characterised by presence of
• Paracetamol With/without codeine in addition to drugs above, solely for • Uricosurics e.g. Sulfinpyrazone, work by increasing renal urate excretion
negatively birefringent crystals under polarised light microscopy
pain relief

234 / Chapter 7 Rheumatology: Gout Mind Maps for Medicine \ 235

 • OA occurs when there is an chondrocytes therefore disrupting Systemic Symptoms • Joint deformity and instability
imbalance between joint the normal cartilage repair • Squaring of the thumb
breakdown and sufficient repair mechanism • Age Risk increases with age • Joint pain: typically worse on • Swelling of the proximal
process • Certain cytokines, e.g. IL-1 and • Gender Polyarticular OA is more common in women movement, load bearing and at interphalangeal (PIP;
Osteoarthritis (OA) is the most common • Normal articulating cartilage TNF-alpha, as well as protease • Family history 40–60% of ‘common OA’ is thought to have the end of the day Bouchard’s nodes) and distal
form of arthritis and is the major cause (hyaline cartilage) undergoes enzymes, e.g. metalloproteinase, a hereditary component • Joint stiffness: typically in the interphalangeal (DIP; Heberden’s
of impaired mobility. It is a condition turnover in which ‘worn out’ are found to be increased in • Bone density Increased bone density, e.g. Paget’s disease, morning or after rest <30 min nodes) joints (see Fig. 1)
characterised by cartilage damage and collagen and other matrix the cartilage of patients with increases risk of OA; reduced bone density, e.g. osteoporosis, • Reduced joint function • Effusion
joint space narrowing resulting in pain, components are degraded and OA triggering direct cartilage reduces risk of OA • Joint instability
functional limitation and impaired quality replaced by chondrocyte cells damage
of life. It can affect any joint but the hip, • Both genetic and environmental • Eventually cartilage destruction Mechanical Signs
knee, lumbar/cervical spine and wrist factors can stimulate apoptosis of exposes underlying bone
• Obesity Places mechanical stress on joint cartilage • Periarticular tenderness
joints are most commonly affected.
• Injury Ligament damage or fractures can lead to abnormal • Crepitus
↓Range of movement
Pathophysiology stress on joint cartilage •
Definition • Joint damage Due to underlying disease, e.g. RA, Paget’s
disease
• Muscle wasting
Fig. 1 Heberden’s nodes (DIP joints)
• Joint site Weight-bearing joints are at increased risk of OA,
(blue arrow on left) and Bouchard’s
e.g. hip and knee joint Clinical features nodes (PIP joints) (red arrow on
• Occupation Cleaners have increased risk of hip, knee and
right) due to osteophyte formation
shoulder OA; hairdressers have increased risk of hand OA;
farmers have increased risk of hip OA

Risk factors
Osteoarthritis

Notes
Osteoarthritis
Investigations

Management • X-ray (LOSS, see Fig. 2):

• Loss of joint space
• Osteophytes
• Subchondral cysts
Non-pharmacological Surgical • Subchondral sclerosis
• Bloods FBC (usually normal),
• Education and advice • Joint replacement: most CRP/ESR (usually normal),
• Exercise commonly hip, knee and base of RF/anti-CCP (negative)
• Weight loss thumb joints; the ankle joint can • MRI Can demonstrate early
• Physiotherapy be fused or replaced thinning of the cartilage
• TENS • Arthroscopy lavage and • Arthroscopy Cartilage loss
• Aids and devices debridement: should only be and erosion
referred if they have OA of the • Joint aspiration May be
Pharmacological Fig. 2 X-ray changes associated
knee with a clear history of considered for swollen joints with OA characterised by the loss
mechanical locking to exclude other causes such
• Paracetamol and/or topical of joint space (arrow), osteophytes
NSAIDs/capsaicin as septic arthritis and gout; and subchondral sclerosis
• Addition of weak opioid e.g. in OA there is sterile viscous
codeine fluid, WCC may slightly elevated
• Oral NSAID + proton pump
inhibitor
• Intra-articular corticosteroid
injections

236 / Chapter 7 Rheumatology: Osteoarthritis Mind Maps for Medicine \ 237

 Primary
Notes
Osteoporosis
Osteoporosis is a progressive systemic • Type 1: postmenopausal Female sex, family history and
skeletal disorder characterised by low • Osteoporosis develops when (most common) ↑Osteoclast SHATTERED:
bone mass and micro-architectural there is excessive bone resorption activity; distal radius and vertebral • Steroid use, Smoking
deterioration of bone tissue with by osteoclast cells at a rate fractures common • Hyperthyroidism Usually asymptomatic unless a
resultant increase in bone fragility that exceeds bone formation by • Type 2: age-related ‘senile’ • Age >50, Alcohol fracture is present:
and susceptibility to fracture. It exists osteoblast cells osteoporosis ↓Osteoblast • Thin (BMI <22) • Vertebral fracture → Back
when bone mineral density (BMD) • This results in decreased bone activity; neck of femur fractures • Testosterone deficiency pain, reduced height, kyphosis,
values are reduced by >2.5 standard mass and incomplete bone common • Early menopause respiratory difficulty
deviations below that observed in remodelling resulting in increased • Renal failure and liver failure • Hip fracture → Painful,
young healthy adults. bone fragility and susceptibility Secondary • Erosive bone disease e.g. RA, shortened and externally rotated
to fracture myeloma hip
Underlying cause present: hormonal, • Deficiency of calcium or • Wrist fracture → Pain and
nutritional, drug related or inherited vitamin D, Diabetes deformity
Definition Pathophysiology
Causes Risk factors Clinical features

Osteoporosis

Management

Conservative measures not tolerated; it is less commonly used as there are increased risks
such as venous thromboembolism (VTE), PE and MI Investigations
• Smoking cessation • Teriparatide A parathyroid hormone analogue; intermittent
• Alcohol reduction exposure activates osteoblasts resulting in new bone formation; Bloods DEXA scan
• Diet: food rich in vitamin D/calcium it is reserved for severe cases
• Weight-bearing exercises • Others: raloxifene (selective oestrogen receptor modulator), • FBC: ↑WCC in inflammatory disease e.g. RA, myeloma The gold standard for diagnosing osteoporosis; dual-energy
• Physiotherapy hormone replacement therapy (HRT) and calcitonin are less • CRP/ESR: ↑ in inflammatory disease e.g. RA, myeloma X-ray absorptiometry is a means of measuring bone mineral
• Reducing fall risk and home assessment commonly used • U&Es: renal failure is a risk factor for osteoporosis density; two scores are calculated:
• Hip protectors • LFTs: liver failure is a risk factor for osteoporosis
Note: The FRAX® tool helps to identify people who may be at risk of
• TFTs: rule out hypo/hyperthyroidism 1. T-score:
Pharmacological developing osteoporosis. It uses risk factors with DEXA measurements
• PTH: ↑ in hyperparathyroidism • Diagnostic of osteoporosis
to estimate 10-year probability of a fracture. It is useful in aiding clinical
• Calcium/vitamin D supplements • Vitamin D: deficiency is a risk factor • Gives the number of standard deviations of the BMD below
decision-making about the use of pharmacological therapies in patients
• Bisphosphonates Usually 1st-line; can be taken once • Calcium: normal that of a young healthy adult
with reduced BMD.
weekly (alendronate), once monthly (risedronate) or • Phosphate: normal • Interpretation:
yearly IV injections (zoledronate) Surgical • ALP: normal • >0 BMD better than reference population
• Denosumab A monoclonal antibody that reduces • FSH: ↑ in menopause • 0 to –1 No evidence of osteoporosis
osteoclast activity which is given by 6-monthly SC • Fixation of fractures or hip replacements (for neck of femur fractures) • Testosterone: to rule out testosterone deficiency in men • –1 to –2.5 Osteopenia
injections; it may be a suitable option in women • Kyphoplasty: balloon or cement for restoration of vertebral height • –2.5 or below Osteoporosis
X-ray • –2.5 or below plus fragility fracture Established
who are unable to comply with instructions for
bisphosphonates osteoporosis
• To confirm suspected fractures 1. Z-score:
• Strontium ranelate Used for the prevention of • Can comment whether bones appear osteopenic but cannot
osteoporotic fractures in postmenopausal women • Compares an individual’s results to others of the same age
determine osteoporosis from X-rays and gender; a Z-score of <–1.5 raises concerns of factors
with osteoporosis but where other medications are
other than ageing and gender contributing to osteoporosis

238 / Chapter 7 Rheumatology: Osteoporosis Mind Maps for Medicine \ 239

 • Both genetic and environmental factors are thought to play a role Symptoms • Weber’s and Rinne’s test: to
• Autosomal dominant inheritance has also been described in elicit possible sensorineural
Notes
Paget’s disease
some families • Paget’s disease is usually hearing loss
• Sequestosome1 (SQSTM1) is the most important gene mutation asymptomatic (70–90%) and • Signs of other complications
involved therefore diagnosed on incidental such as OA and spinal cord
• Mechanical stress and infections from viruses, e.g. abnormal X-ray or biochemical compression
paramyxoviruses, may play a role findings (↑alkaline phosphatase)
Paget’s disease is a common bone • There are thought to be 3 phases involved in the • The mean age of onset is
• Bone pain and deformity
disease characterised by focal pathophysiology: approximately 55 years
• Pathological fractures
increases in bone remodelling • Lytic phase Transient ↑osteoclast activity causing ↑bone • The highest prevalence is in
• ↑Skin temperature over affected
resulting in the abnormal production resorption and marked ↑ in ALP England, USA, Australia and
bone
of bone which is mechanically weak. • Mixed phase Both osteoclastic and osteoblastic activity, with New Zealand; it is rare in Asia,
The most commonly affected bones ↑ levels of bone turnover leading to deposition of structurally Scandinavia and most of Latin Signs
include the pelvis, spine, skull, femur abnormal bone America
and tibia. • Sclerotic phase A chronic sclerotic phase, during which bone • The male to female ratio is • Head signs: ↑skull size, frontal
formation outstrips bone resorption approx. 3:2 bossing, deep-set eyes, large
maxilla with prominent arches
• Bowing of long bones and Fig. 1 Clinical bowing of the tibia
Definition Pathophysiology Epidemiology kyphosis (see Fig. 1) in Paget’s disease
• Increased temperature over
affected bone

Clinical features

Paget’s disease

Investigations
Complications Management
• Blood tests ↑ALP; bone-specific ALP (if known liver disease), phosphate
and calcium are normal (see Table 1)
Common Conservative
Table 1 Blood test results in conditions affecting bone
• Bone pain (most common) • Orthotic devices, sticks and Condition Ca2+ PO43– ALP PTH
• Bone deformity and enlargement: typically walkers may be useful for Paget’s
the pelvis, lumbar spine, skull, femur and disease of the legs → → → →
Osteoporosis
tibia • Adequate intake of calcium and
• ↑Temperature over affected bone due vitamin D
↓ ↓ ↑ ↑
Osteomalacia
to hypervascularity
• Pathological fractures Pharmacological
Paget’s disease → → ↑ →
• 2° OA due to Paget’s disease surrounding
the joint • Bisphosphonates to reduce bone
turnover, e.g. oral risedronate or IV Hypoparathyroidism ↓ ↑ → ↓
• Hearing loss and tinnitus if Paget’s disease
affects the skull bones and compresses the zoledronate
• NSAIDs and paracetamol for pain Pseudohypoparathyroidism ↓ → → →↑
vestibulocochlear nerve
relief
Rarer
Surgical • X-ray Localised enlargement, patchy cortical
• Spinal stenosis thickening with sclerosis, osteolysis and
• Nerve compression syndromes and cauda Surgical procedures include fracture deformity, advancing lytic lesion in the long
equina syndrome fixation (pathological fracture), bones (see Fig. 2)
• Hypercalcaemia joint replacement (secondary OA) • MRI For suspected spinal stenosis and cord
• High-output cardiac failure and osteotomy (deformity) compression
• Paraplegia Fig. 2 X-ray of Paget’s disease
• Osteosarcoma of the femur

240 / Chapter 7 Rheumatology: Paget’s disease Mind Maps for Medicine \ 241
 • The cause of PMR is unknown • Bilateral shoulder or thigh muscle Investigations are essential to support the diagnosis of PMR but also to
• Given the association of PMR with GCA, it is thought that aching pain for ≥1 month rule out any other possible diagnosis:
mechanisms similar to those contributing to GCA may be (see Fig. 1) • Bloods ESR/plasma viscosity/CRP (usually raised but may be
involved • Morning stiffness >45 min normal), FBC, U&Es, LFTs, bone profile (exclude metabolic bone
• Both PMR and GCA are associated with specific alleles of • Age Almost exclusively in people • Systemic features: disease), TFTs (exclude thyroid diseases), protein electrophoresis
Polymyalgia rheumatica (PMR) is an HLA-DR4 aged >50 years; mean age of onset is • Loss of appetite (exclude myeloma), CK normal (exclude polymyositis and
inflammatory condition of unknown • ↑IL-6 in serum and temporal artery biopsy specimens has approx. 73 • Weight loss dermatomyositis), ANA (exclude SLE), RF, anti-CCP (exclude RA)
cause which is characterised by been observed in both PMR and GCA patients, suggesting • Gender Female: male is • Low-grade malaise • Urinalysis
severe bilateral pain and morning an inflammatory role for IL-6 approximately 3:1 • Signs and symptoms of GCA • EMG Normal
stiffness of the shoulder, neck and • The muscles in PMR are histopathologically normal; proximal • GCA Approx. 40–60% of patients with • Depression • US Scan of shoulders and/or hips if diagnosis is unclear; typical
pelvic girdle. Giant cell arteritis (GCA) upper-extremity symptoms in PMR essentially result from GCA have PMR • Prompt response to corticosteroids findings include subacromial bursitis and biceps tendon
is a more serious condition that often glenohumeral synovitis, subacromial bursitis and biceps • Ethnicity Mainly in people of north tenosynovitis and, less frequently, synovitis of the glenohumeral
co-exists with PMR. tenosynovitis, while pelvic girdle symptoms arise from hip European ancestry, although it can Fig. 1 Typical distribution of pain joint or trochanteric bursitis
synovitis and bursitis occur in any ethnic group in patients with PMR

Definition Pathophysiology Risk factors Clinical features Investigations

Polymyalgia rheumatica

Notes
Polymyalgia rheumatica

Management
Differential diagnosis
Glucocorticoids are the mainstay of treatment and result in a
dramatic response: Inflammatory disorders
• Start with a dose of 15–20mg prednisolone
• Clinical response of >70% in 1 week is expected in PMR; • Rheumatoid arthritis
inflammatory markers should normalise within 4 weeks • Spondyloarthropathy
• The dose of prednisolone should be tapered down slowly • SLE
for 3–6 months to a low maintenance level which is • Scleroderma
sustained for a further 6–12 months then gradually reduced • Sjögren syndrome
over the next 6 months with the aim of stopping altogether • GCA
• Due to long-term use of steroids, bone-protective agents, • Dermatomyositis, polymyositis
e.g. bisphosphonates, and gastroprotective agents, e.g. PPIs,
should be co-prescribed Non-inflammatory disorders
• Steroid-sparing agents such as methotrexate and
azathioprine may be used • Degenerative disease: OA, spinal spondylosis
• Patients should be monitored for the emergence of GCA • Rotator cuff disease
• Drug-induced myalgia e.g. statins
• Infections, including viral syndromes, osteomyelitis, tuberculosis
• Paraneoplastic syndromes
• Amyloidosis
• Chronic pain syndromes, fibromyalgia, depression
• Endocrinopathy and metabolic bone disease:
hyper/hypothyroidism, hyper/hypoparathyroidism,
osteomalacia

242 / Chapter 7 Rheumatology: Polymyalgia rheumatica Mind Maps for Medicine \ 243
 • Remains largely unclear but both environmental and genetic Polymyositis Dermatomyositis
factors are likely to play a part in the disease process • Family history
• Involvement of immune mechanisms is supported by the • Female sex (2.5:1) • Proximal symmetrical muscle weakness Presents with above features plus:
Polymyositis (PM) is a rare presence of T cells, macrophages and dendritic cells in the • Age: DM has a bimodal • Muscle pain • Gottron’s papules (see Fig. 1): scaly,
autoimmune connective muscle biopsy of these patients and by the presence of distribution with peaks at 5–15 • Respiratory muscle weakness erythematous eruptions particularly over
tissue disease characterised by autoantibodies, and by HLA-B8 and HLA-DR3 being a strong and 40–60 years; PM mainly • Raynaud’s the extensor surfaces of the MCP, PIP and
inflammation and weakness genetic risk factor occurs at 40–60 and is rare in • Dysphagia DIP joints Fig. 1 Gottron’s papules
primarily of the skeletal muscle. • It is thought to be a T-cell-mediated cytotoxic process directed children • Dysphonia • Heliotrope rash: violet discolouration of
It may however affect other parts against muscle fibres which may be triggered by viruses; • Black people: PM and DM are 3× • Interstitial lung disease e.g. fibrosing the eyelids, occasionally accompanied by
of the body such as the joints, the this results in capillary obliteration and consequent muscle more common in Black people alveolitis or organising pneumonia periorbital oedema (see Fig. 2)
oesophagus, the lungs and heart. infarction leading to weakness of the skeletal muscle than in Caucasians • Systemic features: fever, fatigue and weight • Photosensitivity
When PM pathology extends to • It may be idiopathic or associated with connective tissue • Underlying malignancy loss (due to oesophageal dysmotility) • Nail-fold erythema
the skin, the condition is termed disorders such as SLE • UV light: the rashes in DM often • Macular rash over back and shoulder
dermatomyositis (DM). DM may • DM in patients over the age of 60 years may be suggestive of occur in sun-exposed areas
Fig. 2 Heliotrope rash
co-exist with other connective tissue
disorders.
an underlying systemic malignancy (typically ovarian, breast
and lung cancer)
• Infections: viral infections e.g. HIV,
simian retroviruses, Coxsackie B
Clinical features

Definition Pathophysiology Risk factors

Notes
Polymyositis and dermatomyositis

Polymyositis and
dermatomyositis

Management
Investigations

Non-pharmacological • DMARDs If steroids fail, • Bloods:

azathioprine, cyclophosphamide, • Enzymes CK can be up to 50× higher than upper limit of
• Sun-blocking agents should be methotrexate can be used; for normal; rarely normal in active disease and the level is usually
used for DM lung disease, an aggressive a good indicator of disease activity; levels of other enzymes
• Encourage regular exercise combination regimen may also be raised: ALT, AST, LDH and aldolase
• Physiotherapist and occupational including ciclosporin A or • Inflammatory markers ESR, CRP (may be raised)
therapist involvement tacrolimus + cyclophosphamide • Autoantibodies ANA (+ve in 60%), anti-Mi-2 antibodies
• Speech and language therapist is recommended to be added to (highly specific for DM, but are only seen in around 25%
involvement to help with corticosteroids of patients), anti-Jo-1 antibodies (not commonly seen in
dysphonia and dysphagia • Biological agents TNF-alpha DM – they are more common in PM where they are seen
• Monitor CK levels antagonists, IV immunoglobulin in a pattern of disease associated with lung involvement,
• Screen thoroughly for malignancy and rituximab can be used for Raynaud’s and fever)
in DM and treat treatment of cutaneous DM • Tumour markers To screen for malignancy in older patients
Pharmacological with DM e.g. CA-125 (ovarian), CA-15-3 (breast cancer) and
CA-19-9 (pancreatic malignancy)
• Steroids High-dose prednisolone • EMG Myopathic changes (reduced duration, amplitude and
1st line; dose should be gradually number of action potentials)
reduced according to clinical • Muscle biopsy Confirms diagnosis, shows evidence of
response and CK levels myositis (muscle necrosis, phagocytosis of muscle fibres and
inflammatory infiltrate)
• MRI May show areas of inflammation in muscle

244 / Chapter 7 Rheumatology: Polymyositis and dermatomyositis Mind Maps for Medicine \ 245
 General signs and symptoms Characteristic patterns of psoriatic arthritis (DR SAM)

• The pathophysiology of psoriatic arthritis is • Joint pain and stiffness • DIP joint disease (5–10%) Predominantly DIP
poorly understood • Psoriasis is the strongest risk factor; may occur Typically prolonged morning joint involvement (see Fig. 3); affects men more
• Like other autoimmune conditions, genetically before (70%), after (25%) or at the same time stiffness (>30min), improvement than women; strongly associated with onycholysis
Psoriatic arthritis is a seronegative susceptible individuals are exposed to an as joint symptoms (5%) with use and recurrence with • Rheumatoid pattern (25%) Presentation
inflammatory arthritis affecting environmental trigger (bacteria, stress or • Western White population prolonged rest very similar to RA with symmetrical small joint
the joints and connective tissue entheseal-related peptide) which in turn • Middle age (35–55 years) • Dactylitis or ‘sausage digits’ arthritis affecting the MCP, wrist and PIP joints;
and is associated with psoriasis of activates the immune system • Family history: approximately 40% of individuals (see Fig. 1) distinguishing features are lack of rheumatoid Fig. 3 DIP involvement in
the skin or nails. A variety of joint • This results in T-cell infiltration and with psoriasis or psoriatic arthritis have relatives • Enthesitis Pain, stiffness and Fig. 1 Dactylitis of the toes nodules, RF negative and often presence psoriatic arthritis – highly
patterns are recognised in psoriatic chemokine/cytokine release with psoriasis or psoriatic arthritis; there is an tenderness of insertions into bone of psoriasis characteristic
arthritis, although these • HLA and other genes may determine the exact association between HLA-B27 and psoriatic e.g. the Achilles tendon • Spondyloarthritis (20%) May present with isolated
may overlap. pattern of tissue involvement arthritis • Extra-articular features: sacroiliitis, typical or atypical AS
• Psoriatic skin rash (see Fig. 2) • Asymmetrical oligoarthritis (50%) Large joint
• Nail changes Pitting, inflammatory arthritis often with ankle, knee, wrist
Definition Pathophysiology Risk factors onycholysis, hyperkeratosis or shoulder involvement
• Uveitis • Mutilans arthritis (1–5%) Most rare but severe
Fig. 2 Psoriatic skin rash
form (see Fig. 4); osteolysis results in destruction of
the small joints of the digits with shortening Fig. 4 Hands showing psoriatic

Clinical features arthritis mutilans

Notes
Psoriatic arthritis Psoriatic arthritis

Management Investigations

Bloods
(a)
Non-pharmacological • Intra-articular steroids
Adjunctive therapy; systemic • ESR/CRP Normal Normal
• Physical exercise Helps to steroids at the lowest effective or raised (in active
maintain mobility and reduce Pencil-in-cup
dose may be used but with disease)
stiffness caution • RF/anti-CCP Negative
• Physiotherapy Helps to • Anti-TNF-alpha e.g. • ANA Negative (b)
improve range of motion Adalimumab, etanercept, • Serum IgA Increase
and pain, as well as muscle golimumab and infliximab in about two-thirds of
strengthening of joints should be considered in sufferers
• Surgery Synovectomy and patients with active arthritis • HLA-B27 May aid in
rarely joint replacement and an inadequate response to diagnosis but needs
at least one synthetic DMARD, to be interpreted with
Pharmacological such as methotrexate care
• Ustekinumab Monoclonal
• NSAIDs 1st line for pain relief Imaging Fig. 5 (a) Diagrammatic representation and
antibody directed against
and soft tissue inflammation (b) X-ray (arrows) showing pencil-in-cup
interleukin (IL)-12/23; it can be
• DMARDs 1st line in active • X-ray Soft tissue deformity caused by underlying osteolysis
used alone or in combination
disease and should be swelling in early
with methotrexate for
given at an early stage e.g. disease; erosion of the DIP joint and periarticular new bone
active disease; treatment
methotrexate, sulfasalazine formation, osteolysis and ‘pencil-in-cup deformity’ (see Fig. 5).
with TNF-alpha inhibitors is
or leflunomide • MRI/CT May be more specific and sensitive in picking up subtle signs
contraindicated

246 / Chapter 7 Rheumatology: Psoriatic arthritis Mind Maps for Medicine \ 247
 • Reactive arthritis is thought to be Salmonella • Arthritis Acute, asymmetrical large joint arthritis (often lower limbs), occurring
caused by an infectious trigger, usually a 2–6 weeks after the infection (most often acute, with malaise, fatigue and fever)
bacterial GI or GU infection (see Fig. 1) Yersinia • Lower back pain Due to sacroiliitis and spondylitis
in genetically susceptible individuals GI infection • Enthesitis Plantar fasciitis and Achilles tendinitis

Reactive arthritis
• This leads to immune activation and Shigella • Eyes Uveitis, episcleritis, keratitis and corneal ulcerations
Reactive arthritis is a form of
cross-reactivity with self-antigens • Dactylitis May occur at one or more toes
seronegative spondyloarthritis
causing acute inflammation in the Campylobacter • Urethritis and circinate balanitis (see Fig. 2)
that develops in response to an
affected joint and other tissues • Mouth ulcers
extra-articular infection, typically Fig. 3 Keratoderma blennorrhagica
e.g. enthesis, skin, mucous membranes GU infection Chlamydia • Nail dystrophy and keratoderma blennorrhagica (see Fig. 3)
originating from the gastrointestinal
and eyes approx. 2–6 weeks after the • Reiter syndrome Triad of conjunctivitis, urethritis and reactive arthritis;
(GI) or genitourinary (GU) tract.
initial infection Fig. 1 The key GI and GU bacteria implicated in reactive arthritis
although rare, it follows a GU or GI infection; it can be easily remembered using
It encompasses Reiter syndrome,
• HLA-B27 is positive in most patients the mnemonic ‘can’t see, can’t wee and can’t bend your knee!’ Fig. 2 Circinate balanitis
a term which describes a classic
and not only confers strong risk of reactive arthritis but also predicts the severity and chronicity of the disease (ulcers and vesicles
triad of urethritis, conjunctivitis
surrounding the glans penis)
and arthritis. Risk factors

Definition Pathophysiology

Notes
Reactive arthritis

Reactive arthritis

Management Investigations

• Bloods Raised CRP, ESR, leucocytosis and

Non-pharmacological thrombocytosis (acute phase), ANA, RF and
anti-CCP negative, HLA-B27 positive in 75%,
• Rest and splint affected joint(s) serology for chlamydia
• Physiotherapy • X-ray Normal in early stages; marginal
erosions, plantar spurs, sacroiliitis and
Pharmacological
asymmetrical syndesmophytes may occur in
• NSAIDs For pain relief and soft tissue inflammation chronic cases
• Corticosteroids Intra-articular, for instance sacroiliac joints • Joint aspiration To rule out crystal or septic
can be injected; a short course of oral corticosteroids can be arthritis; synovial fluid is usually sterile and
considered for patients who are unresponsive to NSAIDs or who cloudy with high WCC
develop adverse effects; topical corticosteroids can be used to • Throat, stool, urine culture To identify
treat skin involvement causative organism
• Antibiotics To treat an identified causative organism; • MRI Asymmetrical sacroiliitis and enthesitis
tetracyclines may be useful for chlamydia urethritis (chronic stage)
• DMARDs e.g. Sulfasalazine, methotrexate and ciclosporin may
be used in patients unresponsive to standard treatments

248 / Chapter 7 Rheumatology: Reactive arthritis Mind Maps for Medicine \ 249
 • RA is thought to occur in genetically susceptible • Gender Women are affected 2–4× Articular Extra-articular (see Fig. 2)
individuals who are exposed to an unknown more often than men
environmental antigen resulting in self-stimulation • Genetic susceptibility There are • Stiffness in joints (particularly in the morning and lasts • Eyes Secondary Sjögren syndrome, scleritis and episcleritis, corneal
of the immune system strong associations between HLA-DR4 >1h) ulceration, keratitis
• T cells seem to be the most important cells in the and HLA-DR1 with RA • Symmetrical joint pain • Skin Leg ulcers especially in Felty syndrome (triad of RF-positive RA,
Rheumatoid arthritis (RA) is a immune response and release a variety of pro- • Cigarette smoking A strong risk • Swollen joints neutropenia and splenomegaly), vasculitis rash, nail fold infarcts
common chronic inflammatory inflammatory cytokines including TNF-alpha, IL-1 and IL-6 factor for the development of RA • Small joints of the hand, feet and wrist mainly affected • Rheumatoid nodules Common and may occur in the eyes,
autoimmune disease characterised • B cells further the pathogenic process through antigen • Infection Viral or bacterial infection • Sex Female : male ratio 3:1 subcutaneously, in the lung(s), heart, and less commonly in the vocal cords
by inflammation of the synovial presentation, and autoantibody and cytokine production is a possible trigger for RA • Speed Relatively quick onset over weeks to months • Neurological Peripheral nerve entrapment, atlanto-axial subluxation,
joints leading to a typically • Joint damage begins at the synovial membrane, where • Autoantibodies Rheumatoid factor • Specific hand signs: polyneuropathy, mononeuritis multiplex
symmetrical and occasionally there is an influx and/or local activation of mononuclear (RF) and anti-cyclic citrullinated • Early: swollen MCP, PIP, MTP joints • Respiratory system Pleural involvement (pleurisy, pleural effusion),
deforming peripheral polyarthritis. cells and the formation of new blood vessels causing peptide (anti-CCP) may be present • Later: Boutonnière deformity, swan neck deformity, pulmonary fibrosis, obliterative bronchiolitis, Caplan syndrome (large fibrotic
It also has a wide variety of extra- synovitis and pannus formation; the pannus destroys in the blood prior to the appearance Z-thumb, ulnar deviation (see Fig. 1) nodules with occupational coal dust exposure)
articular manifestations. bone, whereas enzymes secreted by synoviocytes and of arthritis • Cardiovascular system Pericardial involvement, valvulitis and myocardial
chondrocytes degrade cartilage Boutonnière fibrosis, immune complex vasculitis, increased risk of myocardial infarction
deformity • Kidney Rare but includes analgesic nephropathy, amyloidosis
Definition Pathophysiology Risk factors • Liver Mild hepatomegaly and abnormal transaminases are common
• Other: thyroid disorders, osteoporosis, depression, splenomegaly
Swan neck
deformity
Dry eyes Scleritis

Pulmonary Lymphadenopathy
Ulnar fibrosis (uncommon)
deformity
Anaemia
Rheumatoid nodules
Drug-related
Fig. 1 Late specific hand signs of RA ulcers of stomach Hypersplenism (uncommon)

Rheumatoid arthritis
Vasculitic Drug-related renal damage
skin rash
Osteoporosis

Clinical features
Fig. 2 Extra-articular manifestations of RA

Management
Prognosis

A number of features have been shown Conservative Investigations

to predict a poor prognosis in patients
• Regular exercise
with RA:
• Physiotherapy Non-specific Specific
• RF positive
• Smoking cessation
• Poor functional status at presentation • Bloods FBC (normochromic, normocytic anaemia and • RF Positive in 60–70% of patients
• Access to multidisciplinary team: including nurse specialist, physiotherapist,
• HLA-DR4 reactive thrombocytosis are common in active disease), • Anti-CCP Better specificity than RF
rheumatologist, podiatrist and occupational therapist
• X-ray: early erosions (e.g. <2 years CRP/ESR (usually raised but may be normal), U&Es, LFTs • X-ray affected joints (see Fig. 3): soft tissue
• Transcutaneous electrical nerve stimulation (TENS)
from onset) (may have mild elevation of ALP and gamma-GT, also useful swelling, periarticular osteopenia, loss of
• Extra-articular features Medical (see Table 5) as a baseline prior to starting DMARDs), urate, ANA (positive joint space, bony erosions and deformity
• Insidious onset in SLE and related conditions; also in up to 30% of RA)
• Anti-CCP antibodies • NSAIDs • X-ray joint(s) Soft tissue swelling, periarticular osteopenia,
Narrowing of
joint space
• Corticosteroids loss of joint space, bony erosions, deformity (Fig. 3)
• DMARDs • Urinalysis Microscopic haematuria/proteinuria may
Juxta-articular
• Biological agents suggest connective tissue disease bone erosion
• CXR To exclude lung involvement and as a baseline before
Surgical starting methotrexate
• Synovial fluid analysis Exclude septic arthritis or gout if
• A surgical opinion should be sought in some circumstances if the patient does not
diagnosis unclear
respond to non-surgical management Subluxation of Periarticular
proximal phalanx osteopenia
• Surgical procedures include joint prosthesis (e.g. hip and knee), arthroscopy
and tendon reconstruction Fig. 3 Late X-ray features of RA

250 / Chapter 7 Rheumatology: Rheumatoid arthritis Mind Maps for Medicine \ 251
 Rheumatoid arthritis notes

Diagnostic criteria for RA Assessing severity of RA Table 5 (continued)

The American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) criteria are shown in Tables 1 and 2. The Disease Activity Score 28 (DAS28) is a tool used to assess the severity of DMARDs • 1st-line; it is recommended that patients
A comparison of the clinical features of OA and RA is shown in Table 3. RA based on tenderness and swelling at 28 joints (see Fig. 4), ESR and patient’s with newly diagnosed active RA should
self-reported symptom severity (see Table 4). start a combination of DMARDs (including
Table 1 Joint ACR/EULAR criteria (2010) Table 2 ACR criteria (1987)
methotrexate and at least one other
Joint involvement 1. Morning stiffness in or around the joints lasting at least 1h 1 DMARD, plus short-term glucocorticoids)
• The most commonly used DMARDs
1 large joint = 0 0 2. Arthritis of ≥3 joint areas 1 are methotrexate, sulfasalazine and
hydroxychloroquine; others include
3. Arthritis of hand joints (at least one area swollen in wrist, 1 azathioprine, ciclosporin, d-penicillamine,
2–10 large joints 1
MCP or PIP joints leflunomide and mycophenolate mofetil
• Early DMARD treatment is associated with
1–3 small joints 2
better long-term prognosis (ideally within
4. Symmetrical arthritis 1
3 months of onset)
4–10 small joints 3
5. Rheumatoid nodules 1
Biological agents
>10 joints (including at least one small joint) 5
6. Positive RF 1
TNF-alpha • Examples include infliximab, adalimumab
Serology
inhibitors and etanercept
7. Radiographic changes 1
• Blocks the pivotal action of TNF-alpha, a key
Negative RF and anti-CCP 0 Fig. 4 The 28 joints (MCPs, PIPs, wrists, elbows, shoulders and knees) that are examined
Note: criteria 1–4 must be present for at least 6 weeks cytokine in the pathogenesis of RA
to help calculate the DAS28 score
A total score of ≥4 is diagnostic of RA • The current indication for a TNF inhibitor
Low-positive RF or anti-CCP 2
Table 4 Interpretation of DAS28 is an inadequate response to at least two
DMARDs including methotrexate
High-positive RF or anti-CCP 3 Table 3 RA vs OA: clinical features >5.1 High disease activity
Rituximab • An anti-CD20 monoclonal antibody that
Duration of symptoms RA OA 3.2–5.1 Moderate disease activity results in B-cell depletion
• Used in treatment of severe active RA in
<6 weeks 0 RA usually presents symmetrically OA usually presents in <3.2 Low disease activity combination with methotrexate for patients
an asymmetrical joint whose condition has not responded
≥6 weeks 1 <2.6 Remission adequately to other DMARDs (including one
RA usually involves multiple OA more common in larger or more TNF-alpha inhibitors) or who are
Acute-phase reactants small joints joint(s) A decrease by ≤0.6 points or less Poor response intolerant of them

Normal CRP and ESR 0 Pain in RA usually not worsened by OA pain usually worsened A decrease by >1.2 points Moderate or good response Anakinra • An IL-1 receptor antagonist
movement by movement • Used for RA (in combination with
Abnormal CRP or ESR 1 methotrexate) which has not responded to
Table 5 Pharmacological management of RA
Common age of onset of RA is 20–40 Common age of onset of OA methotrexate alone
A total score of ≥6 is diagnostic of RA is >50 NSAIDs • Examples include ibuprofen, naproxen and • On the balance of its clinical benefits and
diclofenac cost effectiveness, anakinra is not currently
RA onset is relatively quick OA onset is typically years • Used for symptomatic relief and also recommended by NICE
(weeks to months) reduced inflammation
• Side effects: bronchospasm in asthmatics, Tocilizumab • An anti-IL-6 receptor monoclonal antibody
RA has extra-articular manifestations OA does not have extra- dyspepsia/peptic ulceration • Indicated for moderate–severe RA (in
articular manifestations combination with methotrexate or alone
Corticosteroids • e.g. Prednisolone if methotrexate is inappropriate), when
RA tends to be worse in the morning OA tends to be worse • Results in rapid reduction in symptom onset response to at least one DMARD or TNF-
after activities and inflammation alpha inhibitor has been inadequate, or in
• Can be given via intra-muscular, intra- those who are intolerant of these drugs
articular and oral routes
• Usually given short term in combination Abatacept • Fusion protein that modulates a key signal
with DMARDs for active disease required for activation of T lymphocytes
which leads to decreased T-cell proliferation
and cytokine production
• It is given via an infusion or SC injection
• Not currently recommended by NICE

252 / Chapter 7 Rheumatology: Rheumatoid arthritis notes Mind Maps for Medicine \ 253
 • Clinical and pathological manifestations of SSc are the Systemic Localised
Scleroderma is an autoimmune result of innate/adaptive immune system abnormalities
connective tissue disorder that leading to production of autoantibodies and cell- Limited/CREST syndrome: Morphea
affects the skin and other organs. mediated autoimmunity
Limited scleroderma affects face and distal limbs • Oval itchy skin patches; waxy and red in
There are two main types: localised • This results in upregulation of certain cytokines, e.g. IL-1,
predominantly (see Fig. 1); CREST syndrome is a appearance
and systemic sclerosis. Localised 4 and 6, which causes connective tissue producing cells
subtype of limited systemic sclerosis: • Does not involve fingers
scleroderma is more common in (namely fibroblasts/myofibroblasts) to produce excessive
• Calcinosis: typically under fingertips • Raynaud’s phenomenon is uncommon
children and is confined to the skin collagen leading to hardening of the tissue
• Raynaud’s phenomenon: usually first sign to • Dilated nailbed capillaries
and subcutaneous tissue. Systemic • Systemic sclerosis pathology and inflammation extends to
show up (see Fig. 2)
scleroderma (SSc) may be limited small blood vessels which results in clinical manifestations
• OEsophageal dysmotility: may present as Linear
(also known as CREST syndrome), of vasculopathy such as Raynaud’s phenomenon, digit
dysphagia or GORD
which accounts for 70% of SSc cases; ulcers, renal crisis and pulmonary hypertension • Positive ANA • Thickened line of skin; ‘knife-like scar’
• Sclerodactyly (stiff fingers) (see Fig. 3)
the remaining 30% of cases are • Family history • Develops in childhood Fig. 2 Raynaud’s phenomenon: transient
• Telangiectasia (dilated small vessels)
diffuse. • Occurs on arm, leg and forehead vasospasm leading to digital hypoxia resulting
Pathophysiology • Female gender (4:1)
• Raynaud’s phenomenon is uncommon in digits changing colour from white → blue
Diffuse: → red; stress and cold are classic triggers
Definition Risk factors • Scleroderma affects trunk and proximal limbs
predominantly (see Fig. 1)
• Sudden and aggressive onset
• Raynaud’s may not present initially
• Telangiectasia
• Hypertension, lung fibrosis and renal
involvement seen
• Poor prognosis

Scleroderma
Limited Diffuse Fig. 3 Build-up of fibrous tissue in the skin
Clinical features can cause the skin to tighten to cause the
Fig. 1 Skin involvement distribution fingers to curl and reduce their mobility in
in systemic scleroderma scleroderma

Notes
Scleroderma

Management
Investigations
• Skin Skin hygiene and use of emollients for dry skin; low-dose
prednisolone or methotrexate if there is associated synovitis
• Raynaud’s 1st-line treatment is a calcium-channel blocker such as
nifedipine, or an angiotensin II receptor antagonist e.g. losartan; other • Bloods FBC (WCC may be raised, anaemia of chronic
options include SSRIs, alpha blockers, statins and phosphodiesterase disease), ESR and CRP (may be raised), LFTs (baseline) and
type 5 inhibitors renal function (scleroderma can cause renal disease)
• GI For GORD see Ch3: Gastro-oesophageal reflux disease; for • Antibodies ANA positive (90%), RF positive (30%), anti-
constipation: dietary fibre and good fluid intake, softening laxatives topoisomerase-1 (scl-70) antibodies associated with diffuse
e.g. lactulose and/or soluble fibre e.g. ispaghula cutaneous systemic sclerosis, anti-centromere antibodies
• Renal disease Treatment of renal crisis is with ACE inhibitors and (ACA) associated with limited cutaneous systemic sclerosis,
dialysis if necessary anti-RNA polymerase 1 and 3 antibodies associated with
• Cardiac For systolic heart failure: immunosuppression with or without diffuse scleroderma (especially with kidney involvement)
a pacemaker, implantable cardioverter defibrillator; ACE inhibitors and • Respiratory Complete pulmonary function tests, CXR,
carvedilol; for diastolic heart failure with preserved LV: diuretics and high-resolution CT chest for suspected interstitial lung
calcium-channel blockers disease
• Respiratory For pulmonary fibrosis: IV cyclophosphamide with or • Cardiovascular ECHO may show raised pulmonary arterial
without mycophenolate mofetil; supportive treatment e.g. oxygen and pressure / hypertension and right ventricular failure
antibiotics (if infection); for pulmonary arterial hypertension: endothelin • GI Barium swallow test for oesophageal dysmotility
receptor antagonists e.g. bosentan, phosphodiesterase type 5 inhibitors
e.g. sildenafil, prostaglandin derivatives e.g. iloprost; supportive
measures include oxygen and diuretics

254 / Chapter 7 Rheumatology: Scleroderma Mind Maps for Medicine \ 255

 • Septic arthritis usually occurs due to the spread of bacteria Gram-positive cocci Joint distribution
from another site in the body to the affected joint(s)
• The commonest route of spread is via the bloodstream • Staphylococcus aureus (most common) • Usually one joint is affected only; however less often more
(e.g. respiratory or urinary tract infections) • Coagulase-negative staphylococci • Prosthetic joint than one can be affected
• It can also occur from direct inoculation e.g. penetrating (more common in prosthetic joints) • Prior joint damage e.g. RA, gout, • Any joint can be affected but the knee is the most common
trauma or local tissue infection e.g. cellulitis and systemic connective tissue disorders site affected overall with the hip most common in children;
Gram-negative cocci this is followed by the shoulder, wrist, elbow and ankle
Septic arthritis is an acute infection osteomyelitis • Diabetes mellitus
(usually bacterial) of a native or • The normal joint has several protective components; • Neisseria gonorrhoeae (more common • Joint surgery
Symptoms/signs (see Fig. 1)
prosthetic joint. Since septic arthritis healthy synovial cells possess significant phagocytic in young adults who are sexually • Penetrating injury
can lead to rapid joint destruction, activity, and synovial fluid normally has significant active) • Low socioeconomic status • Acutely painful joint(s): worse on movement Fig. 1 Septic arthritis of the
immediate accurate diagnosis and bactericidal activity; septic arthritis is more likely if these • Extremes of age (<15 and >55 years) • Swollen joint metacarpophalangeal joints
treatment are essential. Any joint can defence mechanisms are disrupted e.g. by pre-existing Gram-negative bacilli • IV drug use • ↓Mobility of joint
be affected, particularly the lower joint disease, joint surgery or immunodeficiency • Immunodeficiency e.g. HIV • Tender, erythematous and warm joint
limb joints (most commonly hip and • Bacteria are the most common causative pathogens, with • For example E. coli (more common in • Immunosuppression e.g. on • Effusion around joint
knee). viruses and fungi rarely causing septic arthritis diabetics, the elderly and IV drug users) corticosteroids • Systemic features: fever, tachycardia, rash, malaise
• Unable to weight bear on affected side (if lower limb joint affected)
Definition Pathophysiology Causative agents Risk factors • Loosening of implant (chronic infection in prosthetic joint)

Clinical features

Septic arthritis

Notes
Septic arthritis
Investigations

Bloods

• FBC (↑WCC)
Complications Management • CRP/ESR (↑)
• Blood culture: may reveal presence of
microorganisms, organism type and
• Sepsis • Empirical IV antibiotics, e.g. flucloxacillin, sensitivities to antibiotics
• Avascular necrosis while waiting for synovial fluid joint analysis –
• Septic dislocation refer to local guidelines and consultant Imaging
• Chondrolysis microbiologist; antibiotics are usually later
• Shortening of limb switched to oral antibiotics and given for • X-ray affected joint(s): usually normal but may
• Late degenerative change several weeks reveal underlying joint disease
• Orthopaedic review for consideration of • US affected joint: may show presence of
arthrocentesis, lavage and debridement effusion
of joint • CT and MRI affected joint: the most sensitive
• Joint immobilisation with splinting may be methods for diagnosing periarticular
required abscesses, joint effusions and osteomyelitis

Joint aspiration and synovial

fluid analysis

• Gram stain (may show presence of organisms)

• WCC (often raised)
• Culture (reveals organism type and sensitivities
to antibiotics)
• Polarised light microscopy (rule out gout/
pseudogout)

256 / Chapter 7 Rheumatology: Septic arthritis Mind Maps for Medicine \ 257
 • Dry eyes (keratoconjunctivitis sicca)
• SS is much more common in females than • Dry mouth
Sjögren syndrome (SS) is an males (9:1) • ParotiD swelling (see Fig. 1)
autoimmune condition in which • Environmental or endogenous antigens trigger an innate and • SS peaks at age 30–50 years and after menopause • Vaginal Dryness and Dyspareunia
there is lymphocytic infiltration of adaptive immune-induced inflammatory response in genetically • There is a significant overlap between SLE, RA, • Dry cough
exocrine glands, producing the main susceptible individuals scleroderma and SS • Dysphagia
symptoms of xerophthalmia (dry • Biopsies of glandular and extraglandular sites are characterised by • HLA class markers: HLA-DR3, B8, DQ2 and C4 • Systemic features: polyarthritis, arthralgia, Raynaud’s,
eyes), xerostomia (dry mouth) and lymphocytic infiltration allele found in about 50% of Caucasian patients lymphadenopathy, vasculitis, lung, kidney and
enlargement of the parotid glands. • Cellular adhesive molecules, metalloproteinases and neural with SS liver involvement, peripheral neuropathy, myositis, fatigue
The disease is referred to as primary transmitters show alterations in the affected target organs • Family history confers susceptibility
if it develops in isolation and causing fibrosis • A role for viruses, e.g. EBV as an environmental
secondary if it occurs with other • The overlapping clinical features between primary SS and SLE
autoimmune diseases, usually RA, have led to the suggestion primary SS is likely to share similar
trigger, has been suggested but the evidence Clinical features Fig. 1 Bilateral parotid
is mixed swelling in Sjögren
SLE, scleroderma. features to the pathogenesis of SLE
syndrome
Epidemiology and
Definition Pathophysiology risk factors

Notes
Sjögren syndrome
Sjögren syndrome

Complications Management Investigations

• ↑Risk of infection around the • Dry eyes: • Schirmer’s test Filter paper near conjunctival sac
eyes, mouth, the parotid gland • Artificial tears (1st line) to measure tear formation (see Fig. 2)
and vaginal candidiasis • Ciclosporin eye drops • Bloods FBC, CRP/ESR (may be raised), U&Es, LFTs,
• Parotid tumours • Spectacle eye shields anti-Ro (positive in approx. 70% of patients),
• ↑Risk of developing non- • Humidifiers anti-La antibodies (positive in approx. 30%), ANA
Hodgkin’s lymphoma • Dry mouth: (positive in 70%), RF (positive in nearly 100%),
• Encourage oral fluid intake immunoglobulins (hypergammaglobulinaemia),
• Salivary substitutes C4 (low)
• Cholinergic drugs to stimulate • Salivary gland or lip biopsy Shows lymphocyte
secretion of exocrine glands e.g. infiltration
pilocarpine • Lissamine green test and rose bengal eye
• Dry skin Emollients staining May show keratitis
• Dry vagina/dyspareunia Vaginal • Salivary gland scintigraphy Shows decreased
lubricants salivary gland function
• Arthralgia Hydroxychloroquine may • MRI salivary glands May show chronic
be useful sialadenitis

Fig. 2 Schirmer’s test

258 / Chapter 7 Rheumatology: Sjögren syndrome Mind Maps for Medicine \ 259
 • The exact cause of SLE is unknown but multiple factors are associated with the development • HLA markers: HLA-DR2 and HLA-DR3 are • Non-specific symptoms: malaise, fatigue, fever, myalgia,
of the disease, including genetic and environmental factors (see Fig. 1) more common in patients with SLE lymphadenopathy, weight loss
• Defective C4 complement gene: these • Arthralgia: joints and muscle pain, joint swelling,
Genetic susceptibility patients are more likely to develop a lupus-like Jaccoud’s arthropathy (non-erosive arthropathy
HLA-DR2, HLA-DR3, illness characterised by ulnar deviation of the 2nd to 5th
complement levels, • Sunlight exposure due to UV light is thought fingers with metacarpophalangeal joint subluxation)
Autoimmune proliferation Autoantibody • SLE affects approx. 1 in 1000 to be an important environmental factor • Skin rashes: photosensitive rash, malar (butterfly) rash
hormone levels production people in the UK
Hyperactive B-cell/T-cell • Viruses: e.g. Epstein–Barr virus has been (see Fig. 2), discoid rash (see Fig. 3), livedo reticularis
• SLE is more common in women linked (see Fig. 4)
Systemic lupus erythematosus (SLE) is a activation, defective Apoptosis and self-
than men: 10:1 in the age range • Drugs: chlorpromazine, methyldopa, • Serositis: pleuritic, pericarditis
heterogeneous, inflammatory, multisystem Environmental immune complex clearance exposure, self-recognition
18–65 years hydralazine, isoniazid, d-penicillamine and • Raynaud’s phenomenon
autoimmune disease of unknown cause susceptibility UV light and impaired tolerance and cross-reactivity
• SLE is more common in those minocycline are known to cause drug-induced • Non-scarring alopecia
in which antinuclear antibodies (ANA) exposure, microbial of Chinese, Southeast Asian and lupus • Mouth ulcers
are found (often years preceding clinical response African–Caribbean origin • Tobacco smoking: smoking is linked to the • Renal: proteinuria,
symptoms).
• The usual age of onset is development of SLE and also the prognosis haematuria,
Fig. 1 Summary of the pathogenesis of SLE
16–55 years hypertension or a
Definition Risk factors
raised serum urea or
Pathophysiology Epidemiology creatinine
• Neuropsychiatric:
psychosis, seizures,
anxiety/depression Fig. 2 Malar rash

Systemic lupus erythematosus

Fig. 3 Discoid lupus: can Fig. 4 Livedo reticularis

occur in the absence of

Investigations any systemic features

Management Clinical features

Bloods CXR

• FBC Anaemia, leucopenia, • To check for pleural effusion,

Non-pharmacological Pharmacological
thrombocytopenia and rarely infiltrates and cardiomegaly
• Advice about sun exposure and use of • Simple analgesia/NSAIDs: for joint and muscle pancytopenia (if symptomatic)
sunscreen pains • Activated prothrombin time
May be prolonged in patients with X-ray affected joint
• Smoking cessation • Corticosteroids: when simple analgesia and NSAIDs
• Pregnancy and contraception: pregnancy are insufficient to control symptoms or disease antiphospholipids
• ESR Often elevated • Periarticular osteopenia
should be planned; risk of problems with • Hydroxychloroquine: useful for skin lesions, may be present
pregnancy is greatly reduced if disease is well arthralgia, myalgia and malaise • CRP Often normal unless there is
intercurrent infection or serositis
controlled prior to conception; drug therapy • Cyclophosphamide: reserved for treatment of life- MRI
should be reviewed before pregnancy; pills threatening disease, particularly lupus nephritis, • Immunology ANA (99% positive),
that contain oestrogen may exacerbate lupus vasculitis and cerebral disease anti-dsDNA (highly specific), anti- • In suspected CNS lupus
disease or thrombosis and should be used with • Mycophenolate mofetil: can be used to induce Smith (highly specific) positive, RF
caution (barrier methods or progesterone-only remission or as maintenance therapy (positive in 20%), complement C3 ECG
contraception is preferred) • Azathioprine: used as a steroid-sparing agent and C4 (low during active disease)
and C3d (a degradation product) • For all patients with
• Monitoring disease activity: anti-dsDNA • IV high-dose pooled gammaglobulin and
• U&Es May be abnormal in renal cardiopulmonary symptoms
antibody titres, complement levels (↓C3, C4 granulocyte-colony stimulating factor: have
and ↑C3d and C4d suggest increased activity) a role in autoimmune thrombocytopenia and disease
ECHO
ESR, CRP neutropenia
Urinalysis
• Belimumab (cytokine modulator): monoclonal • To investigate pericardial
antibody used as adjunctive therapy in patients • Haematuria, casts (red cell, involvement
with active autoantibody-positive SLE with a high granular, tubular or mixed)
degree of disease activity despite standard therapy or proteinuria

260 / Chapter 7 Rheumatology: Systemic lupus erythematosus Mind Maps for Medicine \ 261
 Systemic lupus erythematosus notes Notes
Systemic lupus erythematosus

SLE diagnostic criteria Antiphospholipid syndrome

Patients can be classified as having SLE if they satisfy 4 of the clinical
or immunological criteria (including at least 1 clinical criterion and 1 Definition
immunological criterion):
Antiphospholipid syndrome is an autoimmune disorder which may be
Clinical criteria associated with SLE but mainly exists as a primary disease. It is an important
cause of recurrent arterial or venous thrombosis and miscarriages. It is
• Acute cutaneous lupus (≥1 of the following) Malar rash, bullous lupus, associated with the presence of antiphospholipid antibodies.
maculopapular rash, photosensitive rash
• Chronic cutaneous lupus (≥1 of the following) Classic discoid rash Clinical features (CLOT)
(localised or generalised), hypertrophic lupus, lupus panniculitis, mucosal
lupus, lupus erythematosus tumidus, chilblains lupus, discoid lupus/lichen • Coagulation defects
planus overlap • Livedo reticularis (see Fig. 4)
• Oral ulcers Palate, buccal, tongue or nasal ulcers • Obstetric (recurrent miscarriage)
• Non-scarring alopecia • Thrombocytopenia
• Synovitis ≥2 joints, characterised by swelling or effusion or tenderness
Diagnosis (1 clinical and 1 lab finding)
in ≥2 joints and ≥30min morning stiffness
• Serositis Pleurisy or pleural effusions or pleural rub • Clinical 1 episode of arterial and/or venous thrombosis or morbidity in
• Renal Persistent proteinuria or red cell casts; characteristic pericardial pain pregnancy
or pericardial effusion or pericardial rub or pericarditis on ECG • Lab Anticardiolipin antibodies or lupus anticoagulant in plasma
• Neurological Seizures or psychosis
• Haemolytic anaemia Management

Immunological criteria Give low-dose aspirin or warfarin for recurrent thromboses. Expert advice
should be sought for pregnancy.
• ANA Above laboratory reference range
• Anti-dsDNA Above laboratory reference range
• Anti-Smith antibodies
• Antiphospholipid antibody
• Low complement levels
• Positive direct Coombs test (in the absence of haemolytic anaemia)

262 / Chapter 7 Rheumatology: Systemic lupus erythematosus notes Mind Maps for Medicine \ 263
 7-dehydrocholesterol DIET
Vitamin D deficiency or • Lack of sunlight • Darker skin: African–Caribbean, Middle Eastern
hypovitaminosis D remains one • Renal disease: due to impairment of C-1 hydroxylation and South Asian
of 25(OH)D • Age: children and elderly UV LIGHT SKIN Ergosterol
of the most common vitamin
deficiencies. It results in inadequate • GI malabsorption: coeliac disease, short bowel • Breastfeeding: mums that are breastfeeding and
mineralisation of bone and clinically syndrome and cystic fibrosis infants who are exclusively breastfed • Normal bone mineralisation depends
Cholecalciferol (vitamin D3) Ergocalciferol (vitamin D2)
manifests as rickets in children and • Liver disease: due to impaired C-25 hydroxylation of • Obesity on adequate calcium and phosphate
osteomalacia in adults. vitamin D • Routine covering of face and hands: common in which is maintained by vitamin D
• Drugs including anticonvulsants, rifampicin, Muslim women who wear veils (see Fig. 1)
cholestyramine, highly active antiretroviral treatment • Housebound: particularly elderly • Vitamin D deficiency is most Liver
(HAART) and glucocorticoids • Sunscreen: skin-concealing garments or strict
Definition • Genetic causes: hypophosphataemic rickets, type 1 sunscreen use
commonly caused by failure
of the kidneys to hydroxylate
(impaired C-1 hydroxylation) and type 2 vitamin D • Pregnancy 25-hydroxyvitamin D (25(OH)D) to Calcidiol 25-hydroxyvitamin D3
resistance rickets (target organ resistance) • Family history of vitamin D deficiency 1,25-dihydroxyvitamin D (1,25(OH)2D)
due to chronic kidney disease and from
Kidney
inadequate UVB sunlight exposure for
Causes Risk factors the formation of vitamin D3 in the skin
• This results in reduced mineralisation Increase
Apart from osteomalacia and rickets, Calcitriol
of bone due to increased PTH in calcium
vitamin D deficiency is also associated 1,25-dihydroxyvitamin D3 Mobilise
response to low circulating levels of and Intestine Bone
with increased risk of the following: + calcium stores
phosphate and calcium phosphorus
• Osteoporosis 24,25-dihydroxyvitamin D3
absorption
• Diabetes mellitus
• Cardiovascular disease
• Cancers such as prostate cancer
Pathophysiology Maintain serum calcium and phosphorus

Complications Vitamin D deficiency

Metabolic functions Bone health Neuromuscular functions
Fig. 1 Vitamin D physiology

Investigations

Management Bloods

• Vitamin D: low
Clinical features
General measures and prevention • Calcium: normal or low
• PTH: usually raised
• Treat underlying cause of vitamin D deficiency • Phosphate: low Rickets
• Adequate sun exposure • ALP: normal or raised Large head
Protruding forehead
• Adequate dietary intake of vitamin D: foods such as oily fish/cod • Infants: growth retardation, hypotonia and apathy
liver oil, egg yolk and milk are rich in vitamin D; some foods are X-rays (infants) Pigeon chest
supplemented with vitamin D, such as breakfast cereals • Once walking: knock-kneed or genu valgum, Depressed ribs Curved humerus
• The following groups are advised to take daily vitamin D tablets Osteomalacia: Fig. 3 Looser zone (arrow) seen in bow-Legged or genu varum, bone deformities Kyphosis
e.g. Adcal-D3: • Pseudofractures the femoral neck of a patient with Enlarged epiphysis at wrist Curved radius and ulna
(see Fig. 2)
• All pregnant and breastfeeding women or Looser zones: osteomalacia • Features of hypocalcaemia (severe vitamin D Protruding abdomen
• All children aged 6 months to 5 years (unless babies are formula pathognomonic of deficiency): paraesthesia, tetany, cramps, seizures
milk fed more than 500ml/day) osteomalacia; they are low-density bands extending from the
Curved femur
• Adults >65 years cortex inwards in the shafts of long bones (see Fig. 3) Osteomalacia
• People who are not exposed to much sun • Coarse trabeculae
• Manage pain symptoms • Osteopenia • Bone pain and tenderness Curved tibia and fibula
• Pathological fractures (particularly femoral neck)
Enlarged epiphysis at ankle
Treatment • Proximal myopathy causing proximal weakness
Rickets:
and potentially a waddling gait
• Vitamin D deficiency requires high-dose replacement; e.g. in adults, • Metaphyseal cupping and flaring Fig. 2 Bone deformities in children with rickets
a daily dose of cholecalciferol 10 000IU or a weekly dose of 60 000IU • Epiphyseal irregularities
will lead to restoration of body stores of vitamin D over 8–12 weeks • Widening of the epiphyseal plates
• Serum vitamin D and calcium levels should be monitored in patients
with vitamin D deficiency after treatment

264 / Chapter 7 Rheumatology: Vitamin D deficiency Mind Maps for Medicine \ 265
 Chapter 8
08
Infectious diseases

Hepatitis A......................................................................................................................................................................268
Hepatitis D and E.......................................................................................................................................................270
Hepatitis B......................................................................................................................................................................272
Hepatitis C......................................................................................................................................................................274
Human immunodeficiency virus.....................................................................................................................276
Malaria..............................................................................................................................................................................280
Tuberculosis..................................................................................................................................................................282

Mind Maps for Medicine \ 267

 • Worldwide, approximately 1.4 million • Travellers to areas with a high prevalence Prodromal phase
cases of HAV are reported every year, • People with clotting factor disorders
but the true incidence is likely to be (Factor VIII and Factor IX concentrates have • Usually lasts 3–10 days
much higher been identified as rare sources of HAV • Flu-like symptoms e.g. general fatigue, malaise,
• HAV is uncommon in the UK and other infection) joint and muscle pain, low-grade fever up to
high-income countries • Men who have sex with men (MSM), and 39°C
• HAV is endemic in many developing people with risky sexual behaviours • GI symptoms including anorexia, nausea,
countries where standards of sanitation • Injecting drug users and their close vomiting, and right upper quadrant abdominal
and food hygiene may be poor e.g. the contacts (at risk of poor standards of pain
Hepatitis A refers to inflammation of Indian subcontinent, sub-Saharan Africa personal hygiene, with possible faecal • May also be headache, cough, sore throat,
the liver caused by infection with the and North Africa, and parts of the Far The mean incubation period contamination of shared drug equipment constipation, diarrhoea, itch or urticaria
hepatitis A virus (HAV). It is the most East, South and Central America and the Transmission of HAV is by the is 28–30 days, with a range and other paraphernalia)
common type of viral hepatitis. Middle East faecal–oral route of 15–50 days • People at occupational risk e.g. laboratory Icteric phase
workers, staff of large residential
institutions, sewage workers, and people • Usually lasts 1–3 weeks, but can persist
Definition Epidemiology Transmission Incubation period who work with primates >12 weeks
• Jaundice, pale stools and dark urine if
cholestasis
• Pruritus
Risk factors • Fatigue, anorexia and nausea/vomiting
• Hepatomegaly, splenomegaly,
lymphadenopathy and hepatic tenderness are
often present on examination
Around 85% of people with HAV infection
make a complete recovery within Convalescent phase
3 months; almost all people with HAV
recover fully within 6 months. • May take up to 6 months
• Malaise, anorexia, muscle weakness and
hepatic tenderness
Prognosis Hepatitis A

Clinical features

Complications Management

Rarely acute liver failure

Acute treatment Immunisation
Investigations
• Mainly supportive e.g. rest, fluids and • An effective vaccine is available; after
anti-emetics initial dose, give booster 6–12 months
• Avoid alcohol until LFTs normalise later
• Admit patients with severe systemic • Indications for vaccination include: Specific antibody tests LFTs and other tests
upset or intractable vomiting for • People travelling to or going to reside
rehydration and observation in areas of high or intermediate • IgM antibody to HAV Positive with onset of symptoms • Alanine transaminase (ALT) rises more than aspartate transaminase (AST) with onset
• Advise pregnant women of increased risk prevalence (if aged >1 year) (it is sensitive and specific); it usually remains positive for of symptoms; levels usually return to reference ranges over several weeks but can
of miscarriage and premature labour and • People with chronic liver disease between 3 and 6 months (up to 12 months) and remains remain elevated for months
need to seek medical advice if symptoms • Patients with haemophilia positive in relapsing hepatitis • Alkaline phosphatase (ALP) rises with ALT and AST
develop • Men who have sex with men • IgG antibody to HAV Appears soon after IgM; in the • Bilirubin rises soon after increase in ALT/AST levels and may remain very high for
• Employment history should be sought • Injecting drug users absence of IgM it indicates past infection or vaccination several months
so patient can be advised appropriately; • Individuals at occupational risk: rather than acute infection; IgG remains detectable for life • Serum albumin levels may fall
until patients become non-infectious, laboratory worker; staff of large • Prothrombin time (PT) usually remains normal but prolongation is a sign of severe
they should avoid food handling and residential institutions; sewage infection
unprotected sexual intercourse workers; people who work with • FBC: mild lymphocytosis is common; pure red cell aplasia and pancytopenia are very
• Advice on managing outbreaks should primates rare
be sought from UK regional public health • People infected with HIV • Liver imaging, e.g. ultrasound, may rarely be required to exclude other diseases
organisations

268 / Chapter 8 Infectious diseases: Hepatitis A Mind Maps for Medicine \ 269
 Hepatitis D and E notes Notes
Hepatitis D and E

Hepatitis D Hepatitis E

• Hepatitis D (HDV) is a single-stranded RNA virus; it is an incomplete virus • Hepatitis E is a liver disease caused by infection with the hepatitis E virus
that requires hepatitis B (HBV) surface antigen (HBsAg) to complete its (HEV, a non-enveloped RNA virus)
replication and transmission cycle • Epidemiology It is common in Central and South-East Asia, North and West
• Co-infection Refers to hepatitis B and HDV infection at the same time Africa, and Mexico
• Superinfection A HBsAg-positive patient who subsequently develops HDV • Transmission Spread by the faecal–oral route (usually by contaminated
infection; this is associated with high risk of fulminant hepatitis, chronic sewage water)
hepatitis status and cirrhosis • Clinical features Incubation period is around 3–8 weeks; it causes a similar
• Epidemiology Approx. 15–20 million people infected with HBV worldwide disease to HAV and is usually self-limiting; fulminant disease occurs in
are also infected with HDV about 10% of individuals, however, and in pregnancy it carries a significant
• Transmission Transmitted parenterally in a similar fashion to HBV (exchange mortality (about 20%)
of bodily fluids) and patients may be infected with HBV and HDV at the • Diagnosis Serum IgM and IgG anti-HEV can be detected by enzyme-linked
same time immunosorbent assay (ELISA)
• Diagnosis Via reverse polymerase chain reaction of HDV RNA • Management Mainly supportive; a promising vaccine has shown a high
• Management Specific treatments include pegylated interferon-alpha and degree of efficacy against HEV but is not yet in widespread use
liver transplantation (which can be curative); management is otherwise
supportive

270 / Chapter 8 Infectious diseases: Hepatitis D and E notes Mind Maps for Medicine \ 271
 • Injecting drug users and their close contacts Acute infection
• People who change sexual partners frequently, MSM,
and sex workers and their clients • Fever, arthralgia, or a rash (may appear 2 weeks prior to the onset of
• Travellers to high-prevalence areas (common via sexual jaundice, then resolves)
exposure or invasive medical procedures) • Non-specific malaise, fatigue, fever, nausea and poor appetite
• People from a country with a high prevalence • Right upper quadrant abdominal pain
• The prevalence of chronic HBV • Household contacts of people with HBV, including • Jaundice (with dark urine and/or pale stools if cholestasis)
infection in the UK is approx. 0.3% close family and carers
• Accidental inoculation of Note: Acute HBV infection is asymptomatic in almost all infants and children,
• In regions with high prevalence, • Families adopting a child from a country with a high
Hepatitis B is an infectious minute amounts of blood 10–50% of adults, and is especially likely in people with HIV
more than 8% of the population prevalence of HBV
disease of the liver caused or fluid
have chronic HBV infection (e.g. in • People receiving regular blood/blood products
by the hepatitis B virus (HBV). • Sexual transmission Chronic infection
Southeast Asia (excluding Japan), 75 days on average, but can vary (e.g. those with haemophilia) and their carers
It can be acute or chronic. • Vertical transmission
China and sub-Saharan Africa) from 30 to 180 days • People with chronic renal failure or chronic liver disease Often there are no physical signs but, depending on the severity and duration,
• People with an occupational risk e.g. healthcare there may be:
workers, laboratory staff and morticians • Spider naevi
75 days on average, but can vary
Definition Epidemiology Transmission Incubation
from 30 to 180 days period
• Looked-after children and young people, including • Finger clubbing
those living in care homes • Jaundice
• Prison inmates and staff • Hepatosplenomegaly
• Infants born to women with HBV infection • Skin thinning, bruising, ascites, liver flap and encephalopathy (severe cases)
• Hajj and Umrah pilgrims with shaved heads (using
unlicensed barbers)
Clinical features
Risk factors

Hepatitis B

Complications
Investigations

• Fulminant liver failure (1%) Management

• Hepatocellular carcinoma Hepatitis B serology
• Glomerulonephritis
• Polyarteritis nodosa Acute hepatitis B Vaccination of an individual with HBV, individuals receiving • Surface antigen (HBsAg) 1st marker to appear and causes
• Cryoglobulinaemia blood transfusions regularly, end-stage CKD the production of anti-HBs. It usually implies acute disease
• There is no specific treatment for acute hepatitis B Passive immunity patients, prisoners, chronic liver disease patients (if present for 1–6 months) and chronic disease if present for
• Admission to hospital should be arranged if very Specific hepatitis immunoglobulin provides >6 months (i.e. infective)
unwell; treatment is aimed at maintaining comfort and passive immunity which can give immediate Testing for anti-HBs following • Anti-HBs Implies immunity (either exposure or immunisation);
adequate nutritional balance, including replacement of negative in chronic disease
but temporary protection in the event of immunisation
fluids lost from vomiting and diarrhoea exposure. • Anti-HBc Implies previous (or current) infection; IgM anti-HBc
• It is vital to avoid unnecessary medications including • Around 10–15% of adults fail to respond or
appears during acute or recent HBV infection and is present for
paracetamol and anti-emetics; alcohol should be respond poorly to 3 doses of the vaccine
about 6 months; IgG anti-HBc persists
avoided until liver enzymes are within normal range
Active immunity • Testing for anti-HBs is only recommended for
• HbeAg Results from breakdown of core antigen from infected
• The local health protection team should be notified of Preparation those at risk of occupational exposure and
liver cells thus a marker of infectivity
suspected cases of acute viral hepatitis patients with CKD; in these patients anti-HBs
The HBV vaccine in the UK contains HBsAg
levels should be checked 1–4 months after Other investigations
adsorbed onto aluminium hydroxide
Chronic hepatitis B adjuvant and is prepared from yeast cells
primary immunisation
• Interpretation of anti-HBs levels: • Baseline FBC, U&Es, LFTs, clotting screen, HbA1c, TFTs, ferritin
using recombinant DNA technology.
• Pegylated interferon-alpha Reduces viral replication • >100 (adequate response) No further • Screening for HCV, HDV, HIV (high co-infection rates)
in up to 30% of chronic carriers; a better response is testing required; should still receive booster at • Screening for hepatocellular carcinoma through ultrasound
predicted for the following: females, <50 years, low Indications 5 years scans of liver and alpha-fetoprotein
HBV DNA levels, non-Asian, HIV negative, high degree • Children born in the UK as part of the • 10–100 (suboptimal response) • Transient elastography (less invasive) or liver biopsy can be
of inflammation on liver biopsy routine immunisation schedule given at 2, 1 additional vaccine dose should be given used to diagnose cirrhosis
• Other antiviral medications aimed at suppressing viral 3 and 4 months of age • <10 (non-responder) A further vaccine
replication include tenofovir, entecavir and telbivudine • At-risk groups: healthcare workers, IV drug course (i.e. 3 doses again) followed by testing
(a synthetic thymidine nucleoside analogue) users, sex workers, close family contacts

272 / Chapter 8 Infectious diseases: Hepatitis B Mind Maps for Medicine \ 273
 • Drug misuse: injecting drug use is the single most
important reported risk factor
• Blood transfusions: receiving a blood transfusion before
September 1991 in the UK
• Pregnancy and breastfeeding: transmission rate
from mother to child is about 6% (breastfeeding is
considered safe)
• Around 214 000 individuals are • Sexual transmission of HCV is possible but uncommon • Approximately 80% of people
chronically infected with HCV • Needle stick injury: a significant occupational risk factor, do not exhibit any symptoms
Hepatitis C infection is a in the UK • Inoculation of minute amounts of e.g. healthcare workers, police, prison staff etc. • Clinical features of acutely
disease of the liver caused by • It is estimated that around 3% blood or fluid (most common) • Use of poorly sterilised medical and dental equipment symptomatic patients may
infection with the blood-borne (170 million) of the world’s • Sexual transmission as well as infected blood products include fever, fatigue, decreased
hepatitis C virus (HCV). It can population are infected; in • Vertical transmission • Tattooing, ear piercing, body piercing or acupuncture appetite, nausea, vomiting,
cause both acute and chronic parts of Europe and the Indian when performed with unsterile equipment abdominal pain, dark urine,
hepatitis and is a major cause subcontinent the prevalence • Sharing razors or toothbrushes which are contaminated grey-coloured faeces, joint pain
Ranges from 2 weeks to 6 months with blood and jaundice
of liver cancer. might be as high as 5% Transmission

Definition Epidemiology Incubation period Risk factors Clinical features

Hepatitis C

Notes
Hepatitis C

Complications Investigations
Management

Acute hepatitis C Acute infection Prevention

Testing for HCV
• Mortality (very low and estimated to • If acute hepatitis C is suspected a same- There is no effective vaccine against
be 0.1% or less) day assessment should be arranged or hepatitis C, therefore prevention of HCV • Testing for anti-HCV antibodies identifies people who
• Acute fulminant hepatitis (rare), immediate specialist advice should be infection depends upon reducing the risk of have been infected with the virus
affecting <1% of all people with sought exposure which includes the following: • If the test is positive for anti-HCV antibodies, a nucleic acid
HCV infection • The local health protection team of • Safe and appropriate use of healthcare test for HCV ribonucleic acid (RNA) is needed to confirm
suspected cases should be notified injections chronic infection (as approx. 30% of people infected with
Chronic hepatitis C immediately • Safe handling and disposal of sharps and HCV spontaneously clear the infection without the need
waste for treatment and though no longer infected they will still
• Symptoms such as fatigue, anxiety Chronic infection • Provision of comprehensive harm-reduction test positive for anti-HCV antibodies)
and depression can negatively services to people who inject drugs
impact on the person’s quality of life • The management of hepatitis C has e.g. needle exchange programme Other investigations
• Cirrhosis advanced rapidly in recent years resulting • Testing of donated blood for HBV and HCV
• Hepatocellular cancer in clearance rates of around 95% (as well as HIV and syphilis) • Baseline FBC, U&Es, LFTs, clotting screen, HbA1c, TFTs,
• Effects of decompensated liver (interferon-based treatments are no longer • Training of health personnel ferritin
disease include oesophageal varices, recommended) • Prevention of exposure to blood during sex • Screening for HAV, HBV and HIV
ascites, bleeding problems, hepatic • The aim of treatment is sustained • Hand hygiene, including surgical hand • Liver ultrasound is used in people with advanced fibrosis
encephalopathy and death virological response (defined as preparation, hand washing and use of or cirrhosis to screen for hepatocellular cancer
undetectable serum HCV RNA 6 months gloves • Transient elastography can be offered to diagnose cirrhosis
after the end of therapy) • Promotion of correct and consistent use of • Liver biopsy considered in individual cases e.g. to assess
• A combination of protease inhibitors condoms the extent of liver damage from inflammation and
(e.g. daclatasvir + sofosbuvir, or cirrhosis, identify iron overload and exclude other causes of
sofosbuvir + simeprevir) ± ribavirin is used liver damage

274 / Chapter 8 Infectious diseases: Hepatitis C Mind Maps for Medicine \ 275
 • Have a current or former partner who is infected
• HIV binds to CD4 receptors on helper
with HIV
T lymphocytes, monocytes, macrophages
• Men who have sex with men (MSM)
and neural cells
• Female sexual contacts of MSM
• CD4 cells migrate to the lymphoid tissue
• From an area of high prevalence of HIV including
where the virus replicates and then • HIV continues to be a major global public health issue,
many parts of Africa
infects new CD4-positive cells; as the • Sexual activity Through vaginal, anal having claimed over 32 million lives so far
• Have had multiple sexual partners
infection progresses, depletion or impaired or oral sex • However, with better access to effective HIV prevention,
• Have a history of sexually transmitted infections
function of CD4 cells predisposes to the • Vertically From mother to child diagnosis, treatment and care, it has become a
(STIs), hepatitis B or hepatitis C infection
development of immune dysfunction during pregnancy or childbirth, manageable chronic health condition, enabling people
• Have a history of injecting drug use
Human immunodeficiency • AIDS is the advanced stage of HIV infection or with breastfeeding living with HIV to lead long and healthy lives
• Have been raped
virus (HIV) is a retrovirus that when the number of CD4 cells is very low • By inoculation Via a contaminated • There are 2 main types of HIV:
• Have had blood transfusions, transplants, or other
infects and destroys cells (<200cells/µl); when the immune system is needle, instrument, blood/blood • HIV-1 (the predominant type in the UK) is highly
risk-prone procedures in countries without rigorous
of the immune system, in impaired to this extent certain opportunistic product; through direct exposure virulent and found worldwide
procedures for HIV screening
particular the CD4 cells (a class infections and malignancies can develop, of mucous membranes or an open • HIV-2 is found mainly in West Africa but has also
• Occupational exposure such as a needle stick injury
of T lymphocyte, also known such as pneumocystis pneumonia and wound to infected bodily fluids; been reported in Portugal, France and increasingly
as T-helper cells). Kaposi’s sarcoma, respectively or by a human bite in India and South America

Risk factors
Definition Pathophysiology Transmission Epidemiology

Human immunodeficiency virus

Stages of HIV and clinical features

1. Seroconversion illness 3. Persistent generalised

lymphadenopathy (PGL)
• Occurs between 1 and 6 weeks
after infection • Nodes >1cm in diameter at 2 extra-
Complications Management Investigations • Common symptoms are fever, inguinal sites persisting for ≥3 months
malaise, myalgia, pharyngitis, not due to any other cause
headaches, diarrhoea, neuralgia • Biopsy is not advised unless an
Increased risk of opportunistic Conservative Prevention of spread • HIV antibody Usually with both a screening ELISA or neuropathy, lymphadenopathy alternative diagnosis must be excluded
infections and a confirmatory western blot assay; most people and/or a maculopapular as features are non-specific
• Patient education including transmission • Promote lifelong safer sex, barrier develop antibodies to HIV at 4–6 weeks (99% by rash; acute infection may be
• Toxoplasmosis reduction advice and psychological contraception and reduction in the 3 months), thus asymptomatic patients should be asymptomatic 4. Symptomatic infection
• Pneumocystis jirovecii pneumonia support number of partners tested 4 weeks post-exposure after an initial negative • Although antibody tests are
• CMV infection • Non-specific constitutional symptoms
• Contact tracing • Warn heterosexuals about the dangers result, and a repeat test at 12 weeks negative, viral p24 antigen and
• Cryptococcal meningitis develop: fever, night sweats, diarrhoea
• General health promotion advice of sexual tourism/multiple sexual • P24 antigen test Usually positive from about HIV RNA levels are raised in early
• Candidiasis and weight loss
on smoking cessation, alcohol, partners 1–4 weeks after infection with HIV infection
• Aspergillosis • Minor opportunistic infections may
diet and exercise, particularly due • Tell drug users not to share needles; use • HIV viral load HIV RNA or branched DNA assay
• Mycobacterium spp. infection 2. Asymptomatic infection be present e.g. oral candida, oral hairy
to cardiovascular, metabolic and needle-exchange schemes • FBC May show anaemia, thrombocytopenia, ↓WCC
leucoplakia, herpes zoster, recurrent
hepatotoxic risk associated with HAART • Vigorous control of other STIs can reduce with ↓CD4 cell count
Increased risk of malignancies HIV incidence by 40% • CRP/ESR May be ↑
After seroconversion, virus levels are herpes simplex, seborrhoeic dermatitis,
Highly active antiretroviral low (although gradual replication tinea infections
• Strengthen awareness of clinics for STIs • Screening for other infections if clinically appropriate:
• Kaposi’s sarcoma therapy (HAART) (see Notes) continues); CD4 and CD8 lymphocyte • The collective name for the above is
• Reduce unnecessary blood transfusions e.g. TB, hepatitis B, toxoplasma, other STIs
• Anal cancer levels are normal; this situation may the AIDS-related complex (ARC), a
• HIV testing in pregnant women; in • Baseline CXR and cervical smear
• Non-Hodgkin’s lymphoma It is recommended that patients start persist for many years prodrome to AIDS
women with HIV, maternal antiretroviral
• Cervical cancer HAART as soon as they have been therapy, C-section (instead of vaginal), 5. AIDS
diagnosed with HIV neonatal antiretroviral therapy and
bottle feeding (instead of breastfeeding) Severe immunodeficiency and evidence
• Post-exposure prophylaxis after of life-threatening infections and unusual
occupational and sexual exposure also tumours
helps to limit HIV spread

276 / Chapter 8 Infectious diseases: Human immunodeficiency virus Mind Maps for Medicine \ 277
 Human immunodeficiency virus notes Notes
Human immunodeficiency virus

Monitoring in HIV Viral load

• Viral load reflects rates of viral replication and is measured using a
Monitoring of HIV will usually be done in specialist clinics using the CD4 polymerase chain reaction (PCR) test
lymphocyte cell (CD4) count and viral load. • A rising viral load may indicate non-adherence to ART or resistance to one or
more antiretroviral drugs
CD4 count • Viral load ranges from undetectable (<50 copies of viral genome/ml blood)
• In a healthy person not infected with HIV, the CD4 count is usually to over 1 million copies/ml
>500cells/µl • The degree of viral replication is linked to the rate of CD4 decline and
• CD4 counts are variable and can be reduced by common infections, therefore disease progression—when viral load is suppressed through
so overall trends are more important than single values ART, CD4 counts recover and risk of HIV-related opportunistic infections
• People with CD4 counts <200cells/µl are most at risk of HIV-related and cancers declines
opportunistic infections and cancers, but some may not have significant
symptoms Highly active antiretroviral therapy (HAART)
• CD4 counts are the main determinant of when prophylaxis for opportunistic
infections and antiretroviral therapy (ART) is started • HAART involves a combination of at least 3 drugs, typically 2 nucleoside
reverse transcriptase inhibitors (NRTIs) and either a protease inhibitor (PI)
or a non-nucleoside reverse transcriptase inhibitor (NNRTI)
• This combination reduces viral replication and the risk of viral resistance
emerging

Table 1 Highly active anti-retroviral therapy

Nucleoside reverse transcriptase inhibitors

Examples Mechanism of action Side effects

Zidovudine (AZT), abacavir, Inhibits synthesis of DNA by reverse transcription General side effects: peripheral neuropathy
emtricitabine, didanosine, and also acts as DNA terminator Tenofovir: renal impairment and osteoporosis
lamivudine, tenofovir Zidovudine: anaemia, myopathy, black nails
Didanosine: pancreatitis

Non-nucleoside reverse transcriptase inhibitors

Nevirapine, efavirenz Binds directly to and inhibits HIV reverse P450 enzyme interaction (nevirapine induces), hepatic
transcriptase, which prevents HIV from replicating disorders, abdominal pain, rashes

Protease inhibitors

Indinavir, nelfinavir, Acts competitively on HIV protease enzyme, Diabetes, hyperlipidaemia, buffalo hump, central
ritonavir, saquinavir which is involved in production of functional viral obesity, P450 enzyme inhibition
proteins and enzymes

Entry inhibitors

Maraviroc, enfuvirtide Maraviroc binds to CCR5, preventing an Alopecia, arthralgia, diabetes, diarrhoea, dizziness,
interaction with gp41; enfuvirtide binds to gp41, dyslipidaemia, dyspnoea, fever, headache,
also known as a 'fusion inhibitor' and prevents neutropenia, oral ulceration, pancreatitis, peripheral
HIV-1 from entering and infecting immune cells neuropathy

Integrase inhibitors

Raltegravir, elvitegravir, Prevents insertion of HIV DNA into the human GI side effects, headaches, myopathy, rhabdomyolysis
dolutegravir genome

278 / Chapter 8 Infectious diseases: Human immunodeficiency virus notes Mind Maps for Medicine \ 279
 Species of plasmodium that are known to cause malaria in • Humans, who are the intermediate hosts, become
Infected mosquito bites
humans include: infected by the bite of an infected female Anopheles
person and they become
• P. falciparum Most prevalent malaria parasite on the African mosquito
infected
continent and responsible for the majority of malaria deaths • The mosquito injects sporozoites (the infecting agent of
• P. vivax Most common form of malaria parasite outside sub- the parasite) into the blood circulation which circulates
Saharan Africa; it has dormant liver stages which can cause and enters liver cells
‘relapses’ of malaria months or years after the initial infection • The sporozoites divide inside liver cells into a schizont
• P. ovale Found mostly in Africa and the islands of the western containing approx. 30 000 offspring (merozoites) which Liver becomes infected
Pacific; it has dormant liver stages which can cause ‘relapses’ are released into the bloodstream when the schizont
of malaria months or years after the initial infection ruptures
• P. malariae Found in South America, Asia and Africa; • Vivax and ovale malaria also have a dormant
if untreated it can cause lifelong chronic infection (hypnozoite) stage in the liver which may ‘awaken’ and Red blood cells become • In 2018, there were an estimated
• P. knowlesi A malaria parasite of monkeys in Southeast become schizont (months or even years after exposure) infected 228 million cases of malaria worldwide
Malaria is a life-threatening Asia which can cause severe and sometimes fatal illness • Red cells infected with P. falciparum adhere to the
• Most malaria cases and deaths occur in
illness caused by infection in humans endothelium of small vessels and cause vascular
sub-Saharan Africa; however Southeast
of red blood cells by • Mixed infections Infection with more than one species occlusion resulting in severe organ damage (mainly gut,
Asia, eastern Mediterranean, western
Plasmodium spp. parasites. of plasmodium can occur kidney, liver and brain) Mosquito bites infected Pacific and the Americas are areas also
• The parasite can also be transmitted by blood person and that mosquito affected
transfusion, transplacentally, organ transplantation and becomes infected
Definition Causes via the use of improperly cleaned syringes
• Children aged under 5 years are the
most vulnerable group affected by
Fig. 1 Malaria transmission cycle
malaria

Pathophysiology (see Fig. 1)

Epidemiology

Malaria

Complications

• Brain Cerebral malaria (due Management

to P. falciparum) resulting Investigations Clinical features
in reduced consciousness,
confusion, convulsions, coma General • Quinine and doxycycline Quinine + doxycycline or
and eventually death doxycycline alone may be used to treat uncomplicated
• Immediate hospital admission should be falciparum malaria; doxycycline should not be given to Diagnostic Most patients with P. falciparum infection present
• Lungs Acute respiratory in the 1st month or within 6 months of infection.
considered in any of the following: children <12 years
distress syndrome Thick and thin blood films: P. vivax or P. ovale infections commonly present
• Suspected of having severe or • Chloroquine Is used to treat uncomplicated P. malariae,
• Blood Severe anaemia, >6 months after exposure (sometimes after years).
complicated malaria P. ovale and P. knowlesi and most cases of P. vivax malaria but • Thick and thin blood films: repeat bloods should be done after 12–24h and again
spontaneous bleeding and
• Suspected of having falciparum malaria use depends upon patterns of resistance and tolerance after a further 24h in patients with initial negative blood film
coagulopathy, septicaemia
• Is a child, pregnant or older than 65 years • In patients with 3 negative slides over a period of 48–72h, the disease is unlikely Symptoms
• Kidneys Nephrotic • Primaquine Only current effective drug for the eradication
• All others suspected of having malaria should of hypnozoites (dormant parasites which persist in the
syndrome, AKI, • Fever (often recurring), chills and rigors
be discussed with an infectious disease liver after treatment of P. vivax and P. ovale); screening for Rapid diagnostic test (RDT):
haemoglobinuria (‘black • Headache
specialist urgently G6PD deficiency is essential before treatment (as can cause
water fever’) • Another frequently used diagnostic test which detects malaria antigen or • Cough
• Public health should be notified of all haemolysis and be fatal in G6PD-deficient individuals); it is
• Metabolic Hypoglycaemia, enzymes • Myalgia
suspected cases also contraindicated in pregnancy and breastfeeding
metabolic acidosis • Slightly less sensitive and not reliable to detect non-falciparum malaria when • Gastrointestinal upset
• Gastrointestinal Jaundice, Antimalarial drugs compared with good-quality blood films; therefore should not be used as an
Prevention (ABCD) Signs
splenic rupture alternative to blood films
• Artesunate Parenteral artesunate is used to treat • Awareness of risk Travellers should be aware of malaria risk
Other • Fever
severe or complicated malaria areas • Splenomegaly, hepatomegaly
• Quinine Used initially to treat severe or • Bite avoidance Use mosquito repellent, e.g. N,N-diethyl- • FBC: typically reveals thrombocytopenia and anaemia, ↑WCC may also be • Jaundice
complicated malaria if artesunate is unavailable meta-toluamide (DEET), covering arms and legs, sleep under present • ± Abdominal tenderness
• Artemisinin-based combination therapy (ACT) an insecticide-treated net • Glucose (low in severe disease), U&Es (may show ↓Na+ and AKI), LFTs (often
Is used to treat uncomplicated malaria and is the • Chemoprophylaxis Malarone, chloroquine, doxycycline, abnormal)
preferred treatment for mixed infection mefloquine or proguanil • G6PD activity: prior to giving primaquine
• Atovaquone-proguanil (Malarone) Is used to • Diagnosis Be aware of symptoms and seek immediate • In severe cases may also need blood gases, blood cultures, clotting, urine/stool
treat uncomplicated falciparum malaria medical advice if develop malaria symptoms, including up culture, CXR and lumbar puncture
to a year after return from travelling

280 / Chapter 8 Infectious diseases: Malaria Mind Maps for Medicine \ 281
 Primary TB Secondary (post-primary) TB

• The initial infection with M. tuberculosis • If the host becomes immunocompromised

usually occurs in the upper zone of the lung the initial infection may become
producing a lesion called the Ghon focus reactivated; reactivation generally occurs
• The Ghon focus comprises macrophages in the apex of the lungs and may spread • Close contact of TB patient
which have engulfed the bacilli resulting in locally or to more distant sites • Ethnic minority groups: predominantly from South Asia and sub-Saharan
the typical granulomatous lesions with • The lungs remain the most common site Africa
caseating central necrosis surrounded by for secondary TB; extrapulmonary infection • Homeless patients, those with alcohol dependency and other drug misusers
epithelioid cells and Langerhans giant cells may occur in the following areas: • HIV-positive and other immunocompromised patients; patients on
• The Ghon focus is almost always accompanied • Central nervous system (tuberculous immunosuppressant drugs are particularly at risk (e.g. infliximab and
by caseous lesions in the hilar lymph nodes meningitis, the most serious etanercept, azathioprine, ciclosporin) • Worldwide, TB is one of the top 10 causes
Tuberculosis (TB) is an
and the combination of these is known as a complication) • Children and elderly patients of death and the leading cause from a
infection caused by bacteria
Ghon complex • Vertebral bodies (Pott’s disease) • Other conditions: haematological and some solid cancers, long-term single infectious agent (above HIV/AIDS)
of the Mycobacterium
• In immunocompetent people the initial • Cervical lymph nodes (scrofuloderma) steroids, diabetes, end-stage renal disease, silicosis and gastrectomy/ • In 2018, an estimated 10 million people
genus that most commonly
lesion usually heals by fibrosis; those who • Renal jejunoileal bypass fell ill with TB worldwide
affect the lungs.
are immunocompromised may develop • GI tract
disseminated disease (miliary TB)
Risk factors Epidemiology
Definition
Pathophysiology

Tuberculosis

Clinical features
Complications Management Investigations
Systemic features

• Reduced quality of life Non-pharmacological • May be asymptomatic

• Transmission to others • CXR Typically shows patchy or nodular
• Night sweats
• Drug resistance • Care should be coordinated through the local multidisciplinary TB team shadows in the upper zone (see Fig. 1);
• Weight loss
• Post-TB bronchiectasis, COPD and • For all TB cases in the UK, the local public health authority should be notified with miliary TB the CXR may be normal or
• Malaise
aspergilloma (occur in residual lung so that screening and contact tracing (see Notes) can be arranged show miliary shadows throughout the lung
• Fever
cavities) • Patient education, particularly the importance of complying with medical (see Fig. 2)
• Anorexia
• Post-TB cor pulmonale/respiratory therapy • Sputum (at least 3 samples) Ziehl–Neelsen
failure may result from lung cavitation, or auramine staining of a sputum smear may Local features
scarring and fibrosis following Pharmacological demonstrate the presence of acid-fast bacilli;
pulmonary TB in vitro culture of the sputum (e.g. Löwenstein– • Lung/pleura Cough, sputum, haemoptysis,
Anti-TB drugs (see Notes, Side effects of anti-TB drugs) Jensen medium) may take 4–7 weeks to breathlessness, lobar collapse, bronchopneumonia,
• Death
Active TB standard treatment (RIPE): provide a result and a further 3 weeks is hoarseness, chest pain, effusion
Fig. 1 Pulmonary TB
• Rifampicin For 6 months (2 months initiation phase + 4 months continuation required to identify drug sensitivity • Heart/pericardium Pain, arrhythmias, cardiac failure,
phase) • Whole blood interferon-gamma Aids pericarditis
• Isoniazid For 6 months (2 months initiation phase + 4 months continuation diagnosis of TB • Intestine Malabsorption, diarrhoea, obstruction
phase) • Bronchoscopy with washings of the affected • GU tract Haematuria, renal failure, epididymitis,
• Pyrazinamide For initial 2 months (2 months initiation phase) lobes; useful if there is no sputum available salpingitis, infertility
• Ethambutol For initial 2 months (2 months initiation phase) • Biopsy Diagnosis can sometimes be made • Adrenals Adrenal insufficiency
Latent TB: based on the histological demonstration of a • Skin Erythema nodosum, erythema induratum, lupus
The treatment for latent TB is 3 months of isoniazid with pyridoxine and rifampicin caseating granuloma) vulgaris
or 6 months of isoniazid (with pyridoxine). • HIV screening HIV is a big risk factor for TB • Eyes Iritis, choroiditis, phlyctenular keratoconjunctivitis
(see Ch8: Human immunodeficiency virus) • Bones/joints Arthritis, osteomyelitis
Meningeal TB: • Tuberculin test Hypersensitivity to the • Lymphatics Lymphadenopathy, cold abscesses,
Patients with meningeal TB are treated for a prolonged period (at least 12 months) tubercle bacillus develops about 3 weeks sinuses
Fig. 2 Miliary TB
with the addition of steroids. after initial infection; the Mantoux test is • Brain Tuberculoma, meningitis
usually used
282 / Chapter 8 Infectious diseases: Tuberculosis Mind Maps for Medicine \ 283
 Tuberculosis notes Notes
Tuberculosis

Side effects of anti-TB drugs (see Table 1) • Previously unvaccinated, tuberculin-negative new entrants under 16 years
who were born in or who have lived for a prolonged period (at least
Table 1 Side effects of the main anti-TB drugs 3 months) in a country with an annual TB incidence of 40/100 000 or greater
• Healthcare workers
Rifampicin • Discolours body secretions and urine pink • Prison staff
• Induces liver enzymes • Staff of care homes for the elderly
• Flu-like symptoms • Those who work with homeless people
• Elevation of liver transferases and hepatitis
Contraindications
Isoniazid • Peripheral neuropathy: prevent with pyridoxine
(vitamin B6) • Previous BCG vaccination or a past history of TB
• Hepatitis • Positive tuberculin test (Heaf or Mantoux)
• Allergic reactions: skin rash and fever • HIV
• Agranulocytosis • Pregnancy
• Inhibits liver enzymes
Contact tracing
Pyrazinamide • Hyperuricaemia • The Mantoux test should be used to diagnose latent TB in people who are
• Arthralgia and myalgia either household contacts or close work or school contacts (aged ≥5 years)
• Gout of all patients diagnosed with active TB
• Hepatitis (rare) • As the Mantoux test may be positive in patients who have had the bacillus
(BCG vaccine) interferon-gamma testing is the recommended 2nd-line
Ethambutol Eyes: optic neuritis (visual acuity and red-green test for people whose Mantoux testing shows positive results, or instead of
colour perception should be done before Mantoux test in people for whom Mantoux testing may be less reliable
treatment)
Observed therapy

The Bacille Calmette–Guérin (BCG) vaccination • Individualised supportive care may be required for people with clinically or
socially complex needs considered to be at high risk of poor adherence to
The BCG vaccine contains live attenuated M. bovis. It offers limited protection treatment
against TB and leprosy. • Examples of these individuals include the homeless, history of drug misuse;
previous TB treatment; multiple drug resistance TB; prisoners; significant
Administration mental health, memory or cognitive problems
• This may include ‘directly observed therapy’ (DOT), where drug treatment
• Any person being considered for the BCG vaccine must first be given a
is given under the observation of a key worker and the person is observed
tuberculin skin test; the only exceptions are children <6 years who have had
to swallow each dose of medication, or ‘video observed therapy’ (VOT) to
no contact with tuberculosis
improve adherence, reduce the risk of stopping treatment early and reduce
• Given intradermally, normally to the lateral aspect of the left upper arm
drug resistance
• BCG can be given at the same time as other live vaccines, but if not
administered simultaneously there should be a 4-week interval

Indications
• All infants (aged 0–12 months) living in areas of the UK where the annual
incidence of TB is 40/100 000 or greater
• All infants (aged 0–12 months) with a parent or grandparent who was born
in a country where the annual incidence of TB is 40/100 000 or greater; the
same applies to older children but if they are 6 years or older they require a
tuberculin skin test first
• Previously unvaccinated tuberculin-negative contacts of cases of
respiratory TB

284 / Chapter 8 Infectious diseases: Tuberculosis notes Mind Maps for Medicine \ 285
 Appendix: Figure acknowledgements

Chapter 1: Cardiology • Fig. 2 – Reproduced from Cardiology in a Heartbeat

(Vaswani et al.); Licensed under: Public Domain; available
at: http://commons.wikimedia.org/wiki/File:Splinter_
Acute coronary syndrome
hemorrhage.jpg
• Fig. 1 – Adapted from http://medicalnotesonline.blogspot. • Fig. 3 – Reproduced from Cardiology in a Heartbeat (Vaswani
com/2011/01/cardiology-myocardial-infarction-and.html et al.); Licensed under: Creative Commons Attribution-
• Fig. 2 – Reproduced from Cardiology in a Heartbeat (Vaswani Share Alike 4.0 International; additional attribution: Wikimedia
et al.) under the Creative Commons Attribution-ShareAlike 4.0 Commons, Gonzalo M. Garcia; available at: http://commons.
International Licence; reproduced with permission from wikimedia.org/wiki/File:Acopaquia.jpg
Life In The Fast Lane (http://lifeinthefastlane.com). • Fig. 4 – Reproduced from http://simple-cardio.blogspot.
• Fig. 3 – Reproduced from Cardiology in a Heartbeat (Vaswani com/2012/06/peripheral-signs-of-infective.html with permission
et al.) under the Creative Commons Attribution-ShareAlike 4.0 from Professor Sanjay Sharma
International Licence; reproduced with permission from
Life In The Fast Lane (http://lifeinthefastlane.com).
Valvular heart disease
• Fig. 1 – Reproduced from https://ecg.utah.edu with permission
Acute pericarditis
• Fig. 2 – Reproduced from http://learningradiology.com/index.
• Fig. 1 – Reproduced from Cardiology in a Heartbeat (Vaswani htm with permission
et al.) under the Creative Commons Attribution-ShareAlike 4.0 • Fig. 3 – Reproduced from Cardiology in a Heartbeat (Vaswani
International Licence; reproduced with permission from et al.) under the Creative Commons Attribution-ShareAlike 4.0
Life In The Fast Lane (http://lifeinthefastlane.com). International Licence; reproduced with permission from
• Fig. 2 – author’s own Life In The Fast Lane (http://lifeinthefastlane.com).

Atrial fibrillation
Chapter 2: Respiratory
• Fig. 1 – basic trace
Acute respiratory distress syndrome
Heart failure
• Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & Khaw).
• Fig. 1 – author’s own
• Fig. 2 – Reproduced from Anatomy and Physiology: an
introduction for nursing and healthcare (Minett & Ginesi)
Asthma
• Fig. 3 – Reproduced from https://meded.ucsd.edu/clinicalmed/ • Fig. 1 – Reproduced from Anatomy and Physiology: an
heart.html with permission introduction for nursing and healthcare (Minett & Ginesi).
• Fig. 4 – Adapted from https://medmnemonics.wordpress. • Fig. 2 ­– Reproduced from https://radiopaedia.org/articles/lung-
com/2011/03/04/heart-failure-chest-x-ray-signs-2/ hyperinflation-1?lang=us (Case courtesy of Assoc Prof Frank
Gaillard, Radiopaedia.org, rID: 10550) with permission.
Hypertension
• Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & Khaw).
Bronchiectasis
• Fig. 2 – Reproduced from Medicine in a Minute (Vaswani & Khaw). • Fig. 1 – Licensed under: Creative Commons Attribution-
Share Alike 4.0 International; additional attribution: Wikimedia
Infective endocarditis Commons, Gonzalo M. Garcia; available at: http://commons.
wikimedia.org/wiki/File:Acopaquia.jpg
• Fig. 1 – Reproduced from www.slideserve.com/derry/ • Fig. 2 ­– Reproduced from image https://radiopaedia.org/cases/
infective-endocarditis cystic-bronchiectasis-3 (Case courtesy of Dr Bruno Di Muzio,
Radiopaedia.org, rID: 18289) with permission.

Mind Maps for Medicine \ 287

 Chronic obstructive pulmonary disease Chapter 3: Gastroenterology Wilson’s disease Hyperthyroidism
• Fig. 1 – Reproduced from Interpreting Chest X-Rays (Ellis). • Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & Khaw). • Fig. 1 – Reproduced from Medicine in a Minute (Vaswani &
Acute pancreatitis Licensed under Creative Commons Attribution-Share Alike 3.0 Khaw). Licensed under Creative Commons Attribution-Share
Interstitial lung disease Unported; additional attribution: Herbert L. Fred, MD, Hendrik Alike 4.0 International; additional attribution: OpenStax; available
• Fig. 1 – reproduced from Chauhan, et al. (2008) Clinical Picture,
A. van Dijk; available at: https://commons.wikimedia.org/wiki/ at: https://cnx.org/contents/FPtK1zmh@8.108:YhivaL0u@4/
• Fig. 1 – Reproduced from Interpreting Chest X-Rays (Ellis). 372: 54 (DOI: https://doi.org/10.1016/S0140-6736(08)60993-9)
File:Kayser-Fleischer_ring.jpg The-Thyroid-Gland
with permission from Elsevier.
• Fig. 2 – Reproduced from Medicine in a Minute (Vaswani &
Lung cancer Khaw). Licensed under Creative Commons Attribution 2.0
• Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & Khaw). Chronic pancreatitis Chapter 4: Renal Generic; additional attributions: Herbert L. Fred, MD and Hendrik
Licensed under Creative Commons Attribution-Share Alike 3.0 • Fig. 1 –­ Reproduced from https://radiopaedia.org/cases/ A. van Dijk; available at: https://commons.wikimedia.org/wiki/
Unported; additional attribution: James Heilman, MD; available at: pancreatic-calcifications-chronic-pancreatitis (Case courtesy of Dr Nephritic syndrome File:Myxedema.jpg
https://commons.wikimedia.org/wiki/File:LungCACXR.PNG Domenico Nicoletti, Radiopaedia.org, rID: 67773) with permission. • Fig. 1 – Reproduced under the Open Government Licence v3.0 • Fig. 3 – Reproduced from Medicine in a Minute (Vaswani & Khaw).
from www.nhs.uk/conditions/henoch-schonlein-purpura-hsp/ Licensed under Creative Commons Attributions-Share Alike
Pleural effusion Cirrhosis • Fig. 2 – Reproduced from Journal of Intensive Care (2017) 5: 57 3.0 Unported; additional attributions: Jonathan Trobe, M.D. –
• Fig. 1 – Reproduced from Interpreting Chest X-Rays (Ellis). • Fig. 1 – Reproduced from www.luxeclinic.co.uk/ (DOI 10.1186/s40560-017-0251-y) under a CC Attribution 4.0 University of Michigan Kellogg Eye Center; available at: https://
what-is-spider-naevus-and-how-to-treat-it/ International License. commons.wikimedia.org/wiki/File:Proptosis_and_lid_retraction_
• Fig. 2 – Licensed under Creative Commons Attributions-Share from_Graves%27_ Disease.jpg
Pneumonia
Alike 3.0; available at: https://commons.wikimedia.org/wiki/ Nephrotic syndrome
• Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & Khaw). File:Gynecomastia_001.jpg Hypocalcaemia
Licensed under Creative Commons Attribution-Share Alike 3.0 • Fig. 1 – Reproduced under a CC Attribution License 3.0
Unported; additional attribution: Hellerhoff; available at: https:// Germany; additional attribution: Klaus D. Peter; available at: • Fig. 2 – Reproduced from https://what-when-how.com/nursing/
Coeliac disease https://en.wikipedia.org/wiki/Periorbital_puffiness#/media/ endocrine-disorders-adult-care-nursing-part-3/
commons.wikimedia.org/wiki/File:03-01-Infiltrat_Ausgang.png
• Fig. 1 – Adapted from www.glutenfreetherapeutics.com/ File:Oedema.jpg
living-gluten-free/medicine-research/chronic-inflammation • Fig. 2 – Reproduced under the Open Government Licence v3.0 Hypothyroidism
Pneumothorax
• Fig. 2 – Reproduced from Medicine in a Minute (Vaswani & Khaw). from www.nhs.uk/conditions/nail-problems/ • Fig. 1 – Reproduced from Anatomy and Physiology: an
• Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & Khaw). Licensed under Creative Commons Attribution-Share Alike 3.0 • Fig. 3 – Reproduced from Medicine in a Minute (Vaswani & Khaw).
Licensed under Creative Commons Attribution-Share Alike 3.0 introduction for nursing and healthcare (Minett & Ginesi).
Unported; additional attribution: BallenaBlanca; available at: Licensed under CC Attribution License 3.0 Germany; additional
Unported; additional attribution: Karthik Easvur; available at: https://commons.wikimedia.org/wiki/File%3ADiapositiva_1.jpg attribution: Klaus D. Peter; available at: https://commons.
https://commons.wikimedia.org/wiki/File:Pneumothorax_gif_1.gif • Fig. 3 – Reproduced from www.passmedicine.com/review/ wikimedia.org/wiki/File:Xanthelasma.jpg
Polycystic ovary syndrome
• Fig. 2 – Reproduced from Interpreting Chest X-Rays (Ellis). textbook.php?s= with permission. • Fig. 1 – Reproduced from Science Photo Library with permission.
• Notes Fig. (Flowchart of management of spontaneous • Fig. 2 – Reproduced from Clinical Endocrinology (Whitehead &
pneumothorax) – Reproduced from Thorax (2010) 65: 18 with Gastric cancer
Chapter 5: Endocrinology Miell).
permission from BMJ Publishing Group Ltd.
• Fig. 1 – Reproduced from www.nguyenthienhung.com
Acromegaly
Pulmonary embolism
• Fig. 2 – Reproduced from Medicine in a Minute (Vaswani Chapter 6: Neurology
& Khaw). Licensed under Creative Commons Attributions- • Fig. 1 ­– Adapted from www.endotext.org.
• Fig. 2 – Reproduced from Medicine in a Minute (Vaswani & Khaw). Share Alike 3.0 Unported; additional attribution: Thomas • Fig. 2 – Reproduced from Medicine in a Minute (Vaswani & Khaw).
Licensed under CC Attribution 2.0 Generic; additional attributions:
Bell’s palsy
Licensed under the Creative Commons Attribution-ShareAlike 4.0 Habif; available at: https://commons.wikimedia.org/wiki/
International Licence; reproduced with permission from Philippe Chanson and Sylvie Salenave; available at: https:// • Fig. 1 – Reproduced from Medicine in a Minute (Vaswani &
File:Acanthosis-nigricans4.jpg
Life In The Fast Lane (http://lifeinthefastlane.com). commons.wikimedia.org/wiki/File:Acromegaly_prognathism. Khaw). Licensed under CC Attribution-Share Alike 3.0 Unported;
• Fig. 3 – Reproduced from Medicine in a Minute (Vaswani & Khaw). JPEG additional attribution: James Heilman, MD; available at: https://
Gastro-oesophageal reflux disease commons.wikimedia.org/wiki/File:Bellspalsy.JPG
Licensed under Creative Commons Attributions-Share Alike 4.0 • Fig. 3 – Licensed under CC Attribution 3.0 Unported; additional
• Fig. 1 –­ Adapted from https://herniagallbladderwa.com.au/ attributions: Deshpande P1, Guledgud MV1, Patil K1, Hegde U2, • Fig. 2 – Reproduced from www.pinterest.co.uk/
International; additional attribution: Rvahudson; available at:
conditions/hiatal-hernia-treatment-perth Sahni A1, Huchanahalli Sheshanna S2; available from https:// pin/773985885937936621/
https://commons.wikimedia.org/wiki/File:CTA_Chest_With_
Massive_Pulmonary_Embolism_and_Complete_Occlusion.jpg en.wikipedia.org/wiki/Macroglossia#/media/File:Macroglossia_ • Fig. 3 – Reproduced from Mortada et al. (2009) Transplant
Hereditary haemochromatosis with_crenations_along_the_margins_and_loss_of_papillae_ Infectious Disease, 11: 72 (https://doi.org/10.1111/j.1399-
• Fig. 1 – Reprinted by permission from Springer Nature; Brissot on_dorsum_surface_of_the_tongue.png 3062.2008.00353.x) with permission from John Wiley and Sons.
Respiratory failure
et al. (2018) Haemochromatosis. Nat Rev Dis Primers 4, 18016 • Fig. 4 – Reproduced from Clinical Endocrinology (Whitehead
• Fig. 1 – Reproduced from www.indiamart.com/proddetail/
(https://doi.org/10.1038/nrdp.2018.16) & Miell). Extradural haematoma
venturi-mask-3663147912.html.
• Fig. 2 – Reproduced from Anatomy and Physiology in Healthcare
Jaundice Adrenal insufficiency (Marshall et al.).
Sarcoidosis
• Fig. 1 – Reproduced from https://imannooor.wordpress.com • Fig. 1 – Reproduced from Clinical Endocrinology (Whitehead • Fig. 3 – Reproduced from Eureka: Neurology and Neurosurgery
• Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & • Fig. 2 – Adapted from https://medical-dictionary. & Miell). (Collins et al.).
Khaw). Licensed under Creative Commons Attribution-Share thefreedictionary.com/Bilirubin+metabolism
Alike 3.0 Unported; additional attribution: James Heilman,
Cushing syndrome Multiple sclerosis
MD; available at: https://commons.wikimedia.org/wiki/index.
Oesophageal cancer and other causes of dysphagia • Fig. 2 – Reproduced from https://commons.wikimedia.org/wiki/ • Fig. 1 – Adapted from Anatomy and Physiology in Healthcare
php?curid=11520780
• Fig. 2 – Reproduced from Current Clinical Medicine; Sarcoidosis • Fig. 2 – reproduced from The Lancet, 290: 2383, Lagergren, et al. File:CushingsFace.jpg under a CC Attribution 2.5 Generic license. (Marshall et al.).
(DA Culver) (© 2009, 2010 The Cleveland Clinic Foundation, all (2017) (DOI: https://doi.org/10.1016/S0140-6736(17)31462-9) • Fig. 3 – Reproduced from www.reddit. • Fig. 2 – Reproduced from Eureka: Neurology and Neurosurgery
rights reserved) with permission. with permission from Elsevier. com/r/Pathognomonic/comments/1sxyig/ (Collins et al.).
purple_abdominal_striae_1cm_cushings_syndrome/

288 / Appendix: Figure acknowledgements Mind Maps for Medicine \ 289

 Myasthenia gravis • Fig. 2 – Reproduced from Medicine in a Minute (Vaswani & Khaw). • Fig. 3 – Reproduced from Rheumatology: a clinical handbook • Fig. 3 – Reproduced from Rheumatology: a clinical handbook
• Fig. 1 – Reproduced from www.epainassist.com/images/Article- Licensed under CC Attribution 2.0 Generic; additional attribution: (Al-Sukaini et al.). (Al-Sukaini et al.).
Images/Myasthenia-Gravis.jpg with permission. Arthritis Research UK Primary Care Centre, Primary Care Sciences, • Fig. 4 – Reproduced from Rheumatology: a clinical handbook • Fig. 4 – Reproduced from Rheumatology: a clinical handbook
Keele University, Keele, UK; available at: https://commons. (Al-Sukaini et al.). (Al-Sukaini et al.).
wikimedia.org/wiki/File:Tophaceous_gout.jpg
Neurofibromatosis
Scleroderma Vitamin D deficiency
• Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & Osteoarthritis
Khaw). Licensed under CC Attribution-Share Alike 3.0 Unported; • Fig. 1 – Reproduced from Rheumatology: a clinical handbook • Fig. 1 – Adapted from Clinical Endocrinology (Whitehead & Miell).
additional attribution: Accrochoc; available at: https://commons. • Fig. 1 – Reproduced from Medicine in a Minute (Vaswani & (Al-Sukaini et al.). • Fig. 2 – Reproduced from Rheumatology: a clinical handbook
wikimedia.org/wiki/File:NF-1-Tache_cafe-au-lait.jpg Khaw). Licensed under CC Attribution-Share Alike 3.0 Unported; • Fig. 2 – Reproduced from Rheumatology: a clinical handbook (Al-Sukaini et al.).
• Fig. 2 – Reproduced from Medicine in a Minute (Vaswani additional attribution: Drahreg01; available at: https://commons. (Al-Sukaini et al.). • Fig. 3 – Reproduced from Rheumatology: a clinical handbook
& Khaw). Licensed under CC Attribution-Share Alike 4.0 wikimedia.org/wiki/File:Heberden-Arthrose.JPG • Fig. 3 – Reproduced from Science Photo Library with permission. (Al-Sukaini et al.).
International; available at: https://commons.wikimedia.org/wiki/ • Fig. 2 – Reproduced from Medicine in a Minute (Vaswani &
Khaw). Licensed under CC Attribution-Share Alike 3.0 Unported;
File:Cutaneous_neurofibroma_(MedMedicine).jpg
additional attribution: James Heilman, MD; available at: https://
Septic arthritis Chapter 8: Infectious diseases
• Fig. 3 – Reproduced from Medicine in a Minute (Vaswani & Khaw). • Fig. 1 – Reproduced from www.omicsonline.org/mexico/septic-
Licensed under Public Domain commons.wikimedia.org/wiki/File:Osteoarthritis_left_knee.jpg
arthritis-peer-reviewed-pdf-ppt-articles/ under a CC Attribution Tuberculosis
4.0 license.
Paget’s disease • Fig. 1 ­– Reproduced from https://radiopaedia.org/cases/
Stroke
• Fig. 1 – Reproduced from Rheumatology: a clinical handbook pulmonary-tuberculosis-29 (Case courtesy of Assoc Prof Frank
• Fig. 1 – Reproduced from Eureka: Neurology and Neurosurgery Sjögren syndrome Gaillard, Radiopaedia.or g, rID: 8632) with permission.
(Collins et al.). (Al-Sukaini et al.).
• Fig. 1 – Reproduced from Rheumatology: a clinical handbook • Fig. 2 – Licensed under CC Share Alike 4.0; additional attribution:
• Fig. 2 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.). James Heilman, MD; available at: https://en.wikipedia.org/wiki/
(Al-Sukaini et al.).
Subarachnoid haemorrhage • Fig. 2 – Reproduced from Rheumatology: a clinical handbook Miliary_tuberculosis#/media/File:PulmonaryTBCXR.png
• Fig. 2 – Reproduced from Medicine in a Minute (Vaswani & (Al-Sukaini et al.).
Polymyositis and dermatomyositis
Khaw). Licensed under CC Attribution-Share Alike 3.0 Unported;
additional attribution: James Heilman, MD; available at: https:// • Fig. 1 – Reproduced from Rheumatology: a clinical handbook Systemic lupus erythematosus
commons.wikimedia.org/wiki/File:Subarach.png (Al-Sukaini et al.).
• Fig. 1 – Reproduced from Rheumatology: a clinical handbook
• Fig. 2 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.).
(Al-Sukaini et al.).
Subdural haematoma • Fig. 2 – Reproduced from Rheumatology: a clinical handbook
• Fig. 1 – Reproduced from Eureka: Neurology and Neurosurgery (Al-Sukaini et al.).
Psoriatic arthritis
(Collins et al.).
• Fig. 2 – Reproduced from Eureka: Neurology and Neurosurgery • Fig. 1 – Reproduced from Rheumatology: a clinical handbook
(Collins et al.). (Al-Sukaini et al.).
• Fig. 2 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.).
Chapter 7: Rheumatology • Fig. 3 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.).
Ankylosing spondylitis • Fig. 4 – Reproduced from Rheumatology: a clinical handbook
• Fig. 1 – Reproduced from Rheumatology: a clinical handbook (Al-Sukaini et al.).
(Al-Sukaini et al.). • Fig. 5 – Reproduced from Rheumatology: a clinical handbook
• Fig. 2 – Reproduced from Rheumatology: a clinical handbook (Al-Sukaini et al.).
(Al-Sukaini et al.).
• Fig. 3 – Reproduced from Rheumatology: a clinical handbook Reactive arthritis
(Al-Sukaini et al.). • Fig. 1 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.).
Fibromyalgia • Fig. 2 – Reproduced from Rheumatology: a clinical handbook
• Fig. 1 – Reproduced from Rheumatology: a clinical handbook (Al-Sukaini et al.).
(Al-Sukaini et al.). • Fig. 3 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.).
Giant cell arteritis
Rheumatoid arthritis
• Fig. 1 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.). • Fig. 1 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.).
• Fig. 2 – Reproduced from Rheumatology: a clinical handbook
Gout (Al-Sukaini et al.).
• Fig. 1 – Reproduced from Rheumatology: a clinical handbook
(Al-Sukaini et al.).

290 / Appendix: Figure acknowledgements Mind Maps for Medicine \ 291

 Index

4AT, 188 rheumatoid, 13, 102–3, 182–3, 214–15, Circinate balanitis, 249
242–3, 250–3 Circle of Willis, 222–3
Abbreviated mental test score (AMTS), 188 septic, 256–7 Cirrhosis, 16–17, 40–1, 52–3, 72–3, 76–8,
ABCD2 score, 217 Asbestosis, 42–5 90–1, 94–5, 102–3, 104–5, 112–13,
Acanthosis nigricans, 87, 178 Ascites, 9, 14–15, 52, 68–9, 76–8, 87, 104, 170–3, 270–1, 272–3, 274–5
ACE inhibitor, 12–13, 14, 16–17, 22–3, 109, 126–7, 273, 274 Clopidogrel, 6–7, 216–17, 220
116–17, 124, 126, 142, 168 Aspirin, 2–3, 4–6, 68–9, 100–1, 198–200, Cluster headache, 200
Achalasia, 96–9 216–17, 218–20, 262 Coal worker’s pneumoconiosis, 42–4
Acromegaly, 20–1, 50–1, 132–3 Asthma, 30–3, 34–5, 36–9, 56–7, 62–3 Coeliac disease, 80–1, 92–3, 96–7, 102–3,
Acute coronary syndrome (ACS), 4–7, 187 Atrial fibrillation, 10–11, 18–19, 162–3, 264–5
Acute glaucoma, see Glaucoma, acute 164–7, 216–17, 220–1 COMT inhibitors, 210–12
Acute kidney injury, see Kidney injury, acute Autoimmune hepatitis, 68–9, 77, 94 Confusion assessment method (CAM), 188
Acute liver failure, see Liver failure, acute Constrictive pericarditis, see Pericarditis,
Acute pancreatitis, see Pancreatitis, acute Bariatric surgery, 145, 168–9 constrictive
Acute pericarditis, see Pericarditis, acute Barthel index, 221 Corticobasal degeneration, 211
Acute respiratory distress syndrome (ARDS), Bell’s palsy, 182–5 Cortisol, 134–5, 136–7, 148–9, 168–9,
28–9, 54–5, 62–3, 68–9, 70–1 Beta blockers, 2–3, 7, 10–11, 12–13, 14–17, 170–1, 174–5
Addison’s disease, 134–5 18–19, 22–3, 30–1, 52–3, 140–1, 150, CREST syndrome, 254–5
Adrenal insufficiency, 134–5, 156–7, 168–9, 166, 176, 206–7, 212 Cryoglobulinaemia, 122–3, 272
282–3 Bisphosphonates, 40, 48, 100, 155, 156–7, Cystic fibrosis, 34–5, 40–1, 56–7, 74–5,
AIDS, 55, 276–8 228, 238–9, 240, 242 76–7, 173, 264–5
Alpha-1 antitrypsin deficiency, 34–5, 36–9, Blatchford score, 110 Cullen’s sign, 71
72–3, 76–7, 94–5 Bouchard’s nodes, 237 Cushing disease, 136–7
Alpha blockers, 23, 176–7, 254 Bronchiectasis, 34–5, 40–1, 42–3, 54–5, Cushing syndrome, 20–1, 134–5, 136–7
Amantadine, 204, 210–12 62–3, 282–3
Aminophylline, 32, 36–7 Bronchitis, chronic, 36–7 DAS28, 253
Aminosalicylates, 82–4, 106 Bronchodilators, 34–5, 36–7, 40–1, 45, 72–3 DDP4 inhibitors, 74–5, 146–7
Amyotrophic lateral sclerosis, 202 B-type natriuretic peptide, 14–16 De Quervain’s thyroiditis, 160–1, 164–5
Angina, 2–3, 4–7, 12–13, 18–19 Delirium, 166, 186–8
Angiotensin receptor blocker, 14–17, 22–3 Calcitonin, 46, 155–7, 238 Dementia, 186–8
Ankylosing spondylitis, 12–13, 42–3, 56–7, Calcium channel blocker, 2–3, 7, 10–11, 13, Lewy body, 210–11
82–3, 106–7, 228–9 22–3, 150, 206, 254–5 Denosumab, 156–7, 238–9
Anticoagulation, 10–11, 18–19, 126–7, 150, Cancer Dermatitis herpetiformis, 81
201, 220 gastric, 86–7, 98 Dermatomyositis, 86–7, 242–3, 244–5
Antimuscarinics, 210–12 lung, 36–7, 45, 46–8, 56–7, 72–3, 98, Diabetes insipidus, 138–9, 156–7
Antiphospholipid syndrome, 217, 262 134–5, 136–7, 244–5 Diabetes mellitus, 2–3, 4–5, 40–1, 74–5,
Anti-TB drugs, 282–4 oesophageal, 80–1, 96–8 80–1, 90–1, 116–17, 126–7,
Aortic regurgitation, 12 Carbamazepine, 192 140–50
Aortic stenosis, 12, 14–15 Cardiac tamponade, 8–9, 14–15 overview, 140–1
Apomorphine, 210–12 Carpal tunnel syndrome, 118, 133, 161, 234 type 1 management, 142–3
Arrhythmogenic right ventricular Central venous thrombosis, 201 type 2 management, 144–7
cardiomyopathy, 18–19 Child–Pugh score, 78 Diabetic ketoacidosis, 148–9, 166
Arthritis Chronic bronchitis, see Bronchitis, chronic Dialysis, 52, 68, 116, 118–21, 122, 156, 254
psoriatic, 246–7 Chronic pancreatitis, see Pancreatitis, chronic Discoid lupus, 261–2
reactive, 248–9 Chvostek’s sign, 155 Dopamine receptor agonists, 212

Mind Maps for Medicine \ 293

 Dressler syndrome, 4–5, 8 Hiatus hernia, 88–9 Leuconychia, 77, 127 Parkinson’s disease, 210–12 Ramsay Hunt syndrome, 182–4 Thiazolidinediones, 146–7
Dysphagia, 96–9, 100–1, 202, 206–7, Highly active antiretroviral therapy (HAART), Levetiracetam, 192 Peak flow, 30–1 Raynaud’s, 244–5, 254–5, 258–9 Thrombectomy, 218
218–19, 244–5, 254–5, 258–9 264–5, 276–8 Levodopa, 210–12 Peptic ulcer disease, 7, 100–1, 156–7 Reactive arthritis, see Arthritis, reactive Thrombolysis, 60–1, 218–20
Human immunodeficiency virus (HIV), Lewy body dementia, 210–11 Pericardial effusion, 8–9, 14–15, 262 Renal transplant, 116, 118–19, 121 Thyroid eye disease, 166
Emphysema, 36–7, 72–3 276–8, 282–3 Light’s criteria, 53 Pericarditis Respiratory failure, 28–9, 30–1, 34–5, Thyroid storm, 164–6
Encephalitis, 139, 173, 174, 186–7, 191, Hyperaldosteronism, 20–1, 152–3 Lisch nodules, 209 acute, 8–9 36–7, 40–1, 42–3, 50–1, 54–5, 56–7, Thyrotoxicosis, see Hyperthyroidism
201, 210 Hypercalcaemia, 46–7, 64–5, 70–1, 74–5, Livedo reticularis, 212, 261–2 constrictive, 8–9, 14–15, 18–19, 52–3, 60–1, 62–3, 186–7, 196–7, 202, Toxic multinodular goitre, 164–6
Endocarditis, infective, 7, 13, 24–5, 122–3, 100–1, 138–9, 156–7, 186–7, 190–1, Liver failure, acute, 68–9 76–8 206–7, 282–3 Transient ischaemic attack (TIA), 216–17,
216–17 240–1 Löfgren syndrome, 65 Peritoneal dialysis, 52–3, 118–21 Restrictive cardiomyopathy, 8–9, 14–15, 218–19
Entresto, 16–17 Hyperosmolar hyperglycaemic state, 150 Lung cancer, see Cancer, lung Phaeochromocytoma, 20–1, 156–7, 176–7 18–19 Transjugular intrahepatic portosystemic
Epilepsy, 186–7, 190–3, 204–5, 208–9, Hyperparathyroidism, 20–1, 46–7, 118–19, Phenobarbital, 192–3 Rheumatic fever, 8–9, 12 shunt (TIPS), 78, 108
216–17 156–7, 238–9 Malaria, 126–7, 280–1 Phenytoin, 68–9, 155, 192–3, 214–15 Rheumatoid arthritis, see Arthritis, Trigeminal neuralgia, 200
Ethosuximide, 192 Hyperprolactinaemia, 132–3, 158–9, 178–9 Mallory–Weiss tear, 108–10 Pleural effusion, 14–15, 46–8, 52–3, rheumatoid Troisier’s sign, 87
Exophthalmos, 165 Hypersensitivity pneumonitis, 43 Membranoproliferative glomerulonephritis, 54–5, 70–1, 78, 126–7, 160–1, Rickets, 264–5 Trousseau’s sign, 155
Extradural haematoma, 194–5, 201 Hypertension, 2–3, 4–7, 10–11, 14–15, 122–3, 126–7 250–1, 260–2 Rockall score, 110 Tuberculosis (TB), 54–5, 134–5, 242–3,
20–3, 36–7, 50–1, 118–21, 122–5, Membranous glomerular disease, 126–7 Pneumonia, 28–9, 30–1, 34–5, 42–3, 282–4
Faecal calprotectin, 83, 93, 106 126–7, 132–3, 136–7, 142–3, 152–3, Meningitis, 139, 174, 183, 186–7, 190–1, 46–7, 52–3, 54–5, 56–7, 62–3, Sarcoidosis, 18–19, 42–3, 52–3, 56–7, Type 1 diabetes mellitus, 140–3, 148–9
Fibromyalgia, 230–1, 242–3 176–7, 182–3, 194–5, 218–21, 222–3, 201, 224, 276, 283 121, 150, 173, 186–7, 196–7, 64–5, 96–7 Type 2 diabetes mellitus, 140–1, 144–7, 150
Focal segmental glomerulosclerosis, 126–7 234–5, 260–1 Mesothelioma, 45, 48, 52–3, 173 218–19, 244, 276–7 Scleroderma, 96–7, 242–3, 254–5, 258–9
Hyperthyroidism, 14–15, 150, 156–7, 160–1, Metformin, 142, 145–7, 178–9 Pneumothorax, 56–8, 62–3 Septic arthritis, see Arthritis, septic Ulcerative colitis, 82–4, 106–7
Gastric cancer, see Cancer, gastric 164–6, 186–7, 238–9 Migraine, 198–201, 216–17 Polycystic ovary syndrome (PCOS), 140–1, SGLT-2 inhibitors, 146–7, 148–9 Upper gastrointestinal bleed, 76–7, 108–10
Gastro-oesophageal reflux disease (GORD), subclinical, 166 Mikulicz syndrome, 65 178–9 Silicosis, 42–5, 282–3 Urethral syndrome, 128–9
88–9, 254–5 Hypertrophic obstructive cardiomyopathy Miller Fisher syndrome, 197 Polymyalgia rheumatica (PMR), 232–3, Sjögren syndrome, 34–5, 74–5, 102–3, Urinary tract infection, 116–17, 128–9,
Giant cell arteritis, 200, 232–3 (HOCM), 12–13, 18 Minimal change glomerular disease, 126–7 242–3 182–3, 242–3, 250–1, 258–9 256–7
Glaucoma, acute, 201 Hypocalcaemia, 70–1, 116–17, 154–5, Mitral stenosis, 13, 18–19 Polymyositis, 242–3, 244–5 Sodium valproate, 70–1, 192 Ursodeoxycholic acid, 102–3, 104–5
Gliptins, see DDP4 inhibitors 190–1, 264–5 Monoamine oxidase B inhibitors, 212 Polyneuropathy, 118–19, 214–15, 250–1 Spider naevi, 76–7, 109, 273
Goodpasture syndrome, 116–17, 118–19, Hypoglycaemia, 168–9, 174–5, 186–7, Motor neurone disease (MND), 202–3 Portal hypertension, 76–8, 104–5 Spirometry, 30–1, 36–8, 40–1, 42–3, Vascular parkinsonism, 211
122–3 190–3, 214–15, 216–17, 280–1 MRC dyspnoea scale, 38 Post-streptococcal glomerulonephritis, 62–3, 72–3, 196–7 Vitamin D deficiency, 118–19, 154–5,
Gout, 234–5, 236–7, 250–1, 256–7 Hypogonadism, 158–9, 174–5 Multiple sclerosis (MS), 98, 173, 182–3, 116–17, 122–4 Spironolactone, 13, 14–17, 22–3, 78, 156–7, 238–9, 264–5
Glasgow Imrie criteria, 70 Hyponatraemia, 23, 46–7, 116–17, 170–3, 204–5 Pretibial myxoedema, 165 152, 178 Wegener’s granulomatosis, see
GLP-1 analogues, 145, 146–7 190–2 Multiple system atrophy, 211 Primary lateral sclerosis, 202 Statin, 2–3, 4–7, 118–19, 140–2, 144, Granulomatosis with polyangiitis
Glucocorticoids, 82–3, 156, 174–5, 242, Hypopituitarism, 90–1, 132–3, 134–5, Myasthenia gravis, 62–3, 98, 164–5, 206–7 Primary polydipsia, 138–9 216–17, 220 Wilson’s disease, 76–7, 94, 112, 210–11
253, 264 158–9, 160–1, 174–5 Myasthenic crisis, 207 Primary sclerosing cholangitis, 76–7, Status epilepticus, 190–3
Granulomatosis with polyangiitis, 125, Hypothyroidism, 8–9, 50–1, 52–3, 102–3, Myxoedema coma, 162 82–4, 94–5, 102–3, 104–5, 106–7 Stroke, 216–17, 218–21 Xanthelasma, 3, 77, 127
52–3, 122–3 158–9, 160–2 Progressive bulbar palsy, 202 Subarachnoid haemorrhage, 174–5, 201,
Graves’ disease, 164–5 subclinical, 162 Nephritic syndrome, 122–5 Progressive muscular atrophy, 202 218–20, 222–3 Yellow nail syndrome, 35
Grey Turner’s sign, 71 Nephrotic syndrome, 14–15, 52–3, 78, 112, Progressive supranuclear palsy, 211 Subdural haematoma, 201, 224–5
Gynaecomastia, 17, 23, 77, 109, 165 Idiopathic pulmonary fibrosis, 42–4, 56–7 126–7, 152–3, 172, 280–1 Pseudobulbar palsy, 202 Sulfonylureas, 145, 146–7, 168–9 Zollinger–Ellison syndrome, 100
IgA nephropathy, 122–3 Neurofibromatosis, 176–7, 208–9, 222–3 Pseudogout, 234–5, 256–7 Syndrome of inappropriate ADH secretion
Haemochromatosis, hereditary, 90–1 Infective endocarditis, see Endocarditis, New York Heart Association (NYHA) Psoriatic arthritis, see Arthritis, psoriatic (SIADH), 172–3
Haemodialysis, 68–9, 118–19, 121, 156–7 infective Classification, 15 Pulmonary embolism, 14–15, 52–3, Systemic lupus erythematosus (SLE), 260–2
Haemolytic uraemic syndrome, 123 Inflammatory bowel disease, 82–4, 106–7 Nitrates, 2–3, 4–7, 13, 14–17 60–1, 62–3, 121, 166, 218–19
Headaches, 20–1, 46–7, 174–5, 190–1, Insulinoma, 168–9 Pulmonary hypertension, 13, 36–7, 50–1, Tension headache, 200
198–201, 224–5, 230–1, 276–7 Interstitial lung disease, 42–5, 56–7, 64–5, Obstructive sleep apnoea (OSA), 14–15, 62–3, 254–5 Thiazide diuretics, 14–15, 17, 22–3, 74–5,
Heart failure, 4–7, 10–11, 12–13, 14–17, 244–5 50–1, 62–3, 132–3, 216–17 138–9, 156–7, 173
18–19, 20–1, 52–3, 60–1, 64–5, 68–9, Irritable bowel syndrome, 80–1, 92–3 Oesophageal cancer, see Cancer,
90–1, 116–17, 118–19, 132–3, 147, Ivabradine, 17 oesophageal
160–1, 164–6, 170–3, 186–7, 216–17, Oesophagitis, 88–9, 98–9, 108–9
218–19, 254–5 Jaccoud’s arthropathy, 261 Osteoarthritis, 112, 230, 236–7
Heberden’s nodes, 237 Jaundice, 68–9, 70–1, 76–7, 86–7, 94–5, Osteomalacia, 80–1, 82–3, 118–19, 240–1,
Heerfordt syndrome, 65 102–3, 104–5, 108–9, 166, 268–9, 242–3, 264–5
Henoch–Schönlein purpura (HSP), 122–3 272–3, 274–5, 280–1 Osteoporosis, 102–3, 106–7, 136–7, 156–7,
Hepatic encephalopathy, 68–9, 76, 78 158–9, 164–6, 228–9, 232–3, 238–9,
Hepatitis A, 68–9, 90–1, 94–5, 268–9 Kartagener’s syndrome, 34–5 240–1, 250–1, 264–5
Hepatitis B, 116–17, 122–3, 126–7, 270, Keratoderma blennorrhagica, 249
272–3, 276–7 Kidney injury, acute, 54–5, 76–7, Paget’s disease, 236–7, 240–1
Hepatitis C, 68–9, 274–5 116–17, 122–3, 128–9, 148–9 Pancreatitis
Hepatitis D, 270 acute, 28–9, 70–1, 154–5
Hepatitis E, 270 Lamotrigine, 192 chronic, 70–1, 74–5
Hereditary haemochromatosis, 90–1 Lateral medullary syndrome, 218–19 Parkinsonism, 112–13, 210–12

294 / Index Mind Maps for Medicine \ 295

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 Definition Causes / risk factors
MIND MAPS FOR MEDICINE
Symptoms

MIND MAPS FOR MEDICINE

An innovative, visual textbook to help medical students learn
and understand core medical conditions. Signs

The book features over 100 easy to follow, All mind maps are presented consistently
full colour mind maps of clinically relevant and cover: Pathophysiology
medical conditions using a systems-based • Definition Clinical features
structure: • Pathophysiology
• Cardiology • Causes and risk factors
• Respiratory • Clinical features: signs and symptoms
• Gastroenterology • Epidemiology
• Renal • Investigations: blood tests and imaging
• Endocrinology • Management: lifestyle, pharmacological
• Neurology and surgical
• Rheumatology • Complications
• Infectious diseases

MIND MAPS
Other key features:
The mind maps give you quick access to • Images are provided throughout the
key information in a visually appealing book to help illustrate key signs.
way. Where appropriate the mind map • Mnemonics are used throughout to
is followed by additional reference aid learning.

FOR MEDICINE
information to remind you about, for • Information is up-to-date and based
example, risk assessment tools, staging around the latest guidelines.
criteria, and treatment algorithms. • All topics are clinically relevant or likely to
appear in medical school examinations.
Mind Maps for Medicine is crucial reading for all medical students
but particularly those who consider themselves visual learners.

Pre-publication reviews from medical students

“Exactly what has been missing from the medical school textbook scene. It encompasses all
the need-to-know conditions, in a succinct yet detailed way, which makes studying easy.”
Blood tests
“This great book utilises the mind map format to present the core information, with handy
revision aids throughout!” Management

Azam
“Concise, colourful, and easy-to-follow – a great guide to common medical pathologies.” Investigations
“The mind maps are great – easy to follow with just the right amount of detail for medical students.”
Pharmacological
“The level of detail, organisation of information and layout are great and will make studying very
Imaging
enjoyable!! I think the addition of the ‘notes’ areas for many of the conditions is a great feature too!”
Lifestyle Surgical
“These mind maps look great! Absolutely nailed it in terms of level of detail and layout/design”

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ISBN 978-1-911510-36-9
Complications
9 781911 510369
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