Lec: 5 Dr.

Mohammed Alhamdany

Objective:

1- To know definition and causes of bronchiectasis.

2- To know the clinical features of bronchiectasis.
3- To determine the steps in the diagnosis bronchiectasis.
4- To determine the steps in the management of bronchiectasis.
5- To discuss the prognosis and prevention of bronchiectasis.
6- To know the Genetics, pathogenesis and epidemiology of Cystic fibrosis
(CF).
7- To identify the Clinical features and complications of cystic fibrosis
8- To determine the management of Cystic fibrosis.

Bronchiectasis dilatation
Permanent +irreversable
Bronchiectasis means abnormal dilatation of the bronchi. Chronic suppurative
airway infection with sputum production, progressive scarring and lung damage
occur, whatever the cause.

Aetiology

Tuberculosis Regard as most common cause worldwide.

Causes of bronchiectasis

1- Congenital

A- Cystic fibrosis ‫ ﻣﺎﻧﻜﺪر ﻧﺤﺮﻛﮫ ﻟﻸﻋﻠﻰ‬mucus thick ‫ال‬

B- Ciliary dysfunction syndromes:

1- Primary Ciliary dyskinesia (immotile cilia syndrome)

2- Kartagener syndrome (sinusitis and transposition of the viscera)

C- Primary hypogammaglobulinaemia

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2- Acquired:

A- Children Severe infections………‫ﺗﺆدي إﻟﻰ ﺗﻄﻮر اﻟﻤﺮض‬

1- Severe infections in infancy (especially whooping cough, measles)
2- Primary tuberculosis
3- Inhaled foreign body ‫ ﻻن ﯾﺴﺪ اﻟﻤﻜﺎن وﻣﺎﯾﻜﺪر ﯾﻄﻠﻊ اﻟﺒﻠﻐﻢ‬recurrent infections ‫ﯾﺴﻮي‬

B- Acquired: adults

1- Suppurative pneumonia
2- Pulmonary tuberculosis
3- Allergic bronchopulmonary aspergillosis complicating asthma
4- Bronchial tumours

Localised bronchiectasis may occur due to the accumulation of pus beyond an

obstructing bronchial lesion, such as ‫ھﻨﺎ ﻓﻘﻂ ﻣﻜﺎن ﻣﻌﯿﻦ ﻣﺴﺪود‬
‫وﻟﯿﺲ ﻛﻞ اﻟﺮﺋﺔ ﻓﮭﺬا ﯾﺪل اﻛﻮ‬
1- Enlarged tuberculous hilar lymph nodes ‫ورم او ﺟﺴﻢ ﻏﺮﯾﺐ ﺳﺪﻟﻲ ھﺬا‬
2- A bronchial tumour ‫اﻟﻤﻜﺎن ﺑﺲ وﺑﻘﯿﺔ اﻟﺮﺋﺔ ﻣﺎﺑﯿﮭﺎ‬
‫ﺷﻲ‬
3- An inhaled foreign body.

Clinical features
Chronic cough +copious sputum = bronchiectasis
Symptoms of bronchiectasis

1- Cough: chronic, daily, and persistent

2- Sputum: copious, continuously purulent

3- Pleuritic pain: when infection spreads to involve pleura, or with segmental

collapse due to retained secretions

4- Haemoptysis Streaks of blood common, larger volumes with exacerbations of

infection ‫ ﻓﯿﻜﺢ دم واﻏﻠﺐ اﻷﺣﯿﺎن‬erosion ‫ ﺻﺎﯾﺮ ﺑﻲ‬bronchial artery ‫ﯾﻜﺢ دم ﺑﺴﺒﺐ‬
‫وﺣﺪھﺎ ﺗﺨﺘﻔﻲ ھﺎي اﻟﺤﺎﻟﺔ‬
Massive haemoptysis requiring bronchial artery embolisation sometimes occurs

5- Infective exacerbation: increased sputum volume with fever, malaise, anorexia

6- Halitosis: frequently accompanies purulent sputum ‫رﯾﺤﺔ ﻧﻔﺴﮭﻢ اﺑﺪ ﻣﻮطﯿﺒﺔ‬

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7- General debility: difficulty maintaining weight, anorexia, exertional
breathlessness

Physical signs

In the chest there may be unilateral or bilateral. If the bronchiectatic airways do

not contain secretions and there is no associated lobar collapse, there are no
abnormal physical signs.

When there are large amounts of sputum in the bronchiectatic spaces, numerous
coarse crackles may be heard over the affected areas.

Collapse with retained secretions blocking a proximal bronchus may lead to locally
diminished breath sounds, while advanced disease may cause scarring and
overlying bronchial breathing. ‫اﻟﻤﺮض ھﺬا ﻣﺰﻣﻦ وﻻﯾﻤﻜﻦ اﻟﺸﻔﺂء ﻣﻨﮫ ﻟﮭﺬا‬
prognosis ‫اﻟﻌﻼﺟﺎت اﻟﻲ ﻧﻌﻄﯿﮭﺎ ﻣﺠﺮد ﺗﺄﺧﺮ ﻣﻦ‬
It’s usually causes clubbing of finger. exacerbations ‫وﺗﻘﻠﻞ ﻣﻦ ﺣﺎﻻت‬

Complication

1- Pneumonia.
2- Lung abscess. ‫ ﺑﻤﻜﺎن ﻣﻌﯿﻦ ﻣﻦ اﻟﺮﺋﺔ‬pus ‫ﯾﺘﺠﻤﻊ‬
3- Empyema.
4- Septicemia.
5- Cor pulmonale.
6- Respiratory failure.
7- secondary amyloidosis with nephrotic syndrome

Investigations

1- In addition to common respiratory pathogens, sputum culture may reveal

Pseudomonas aeruginosa and Staphylococcus aureus, fungi such as Aspergillus
and non-tuberculous mycobacteria.

2- Mild bronchiectasis may not be apparent on a plain chest X-ray. In advanced

disease, thickened airway walls, cystic bronchiectatic spaces and associated areas
of pneumonic consolidation or collapse may be visible.

3- CT is much more sensitive and shows thickened, dilated airways.

Confirm test =high resolution CT
‫ﻧﺴﺘﺨﺪﻣﮫ ﻻن ﺣﯿﻀﮭﺮﻟﻨﮫ ﺑﺪﻗﺔ ﻋﺎﻟﯿﮫ اﻟﻘﺼﺒﺎت اﻟﻤﺘﻮﺳﻌﺔ‬
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 Asthma +copd =pulmonary function test ‫ﻧﺴﺘﺨﺪﻣﻠﮭﻦ‬
Bronchiectasis =imaging

Management
‫اﺧﻠﻲ اﻟﻤﺮﯾﺾ ﯾﺴﻮي وﺿﻌﯿﺎت ﻣﻌﯿﻨﮫ واﻟﻮﺿﻌﯿﺔ ﺗﻜﻮن‬
Physiotherapy ‫ﺣﺴﺐ ﯾﺎﻓﺺ ﺑﺎﻟﺮﺋﺔ ﻣﺘﻀﺮر واﺧﻠﻲ ﯾﺘﻨﻔﺲ ﺑﻌﺪﯾﻦ‬
‫ﯾﺤﺎول ﯾﻜﺢ وﯾﻄﻠﻊ اﻟﺒﻠﻐﻢ اﻟﺒﻲ ﻛﻠﮫ‬
Patients should be shown how to perform regular daily physiotherapy to assist the
drainage of excess bronchial secretions. This reduces the amount of cough and
sputum and prevents recurrent episodes of bronchopulmonary infection. Patients
should lie in a position in which the lobe to be drained is uppermost, Deep
breathing, followed by forced expiratory maneuvers (the ‘active cycle of breathing’
technique), helps to move secretions in the dilated bronchi towards the trachea,
from which they can be cleared by vigorous coughing.

The optimum duration and frequency of physiotherapy depend on the amount of

sputum but 5–10 minutes twice daily is a minimum for most patients.

Antibiotic therapy

For most patients with bronchiectasis, the appropriate antibiotics are the same as
those used in COPD but larger doses and longer courses are required. When
secondary infection occurs with staphylococci and Gram-negative bacilli, in

‫إذا ﻣﺮﯾﺾ ﺻﺎرﻋﻨﺪه ﺑﺎﻟﺴﻨﮫ اﻛﺜﺮ‬

4 exacerbation ‫ﺣﺎﻻت‬٣ ‫ﻣﻦ‬
‫ودﺧﻞ ﻟﻠﻤﺴﺘﺸﻔﻰ ﻓﻼزم اﺑﻠﺶ وﯾﺎه‬
antibiotics
 Pseudomonas ‫ ﺳﺒﺒﮭﺎ ﺑﻜﺘﺮﯾﺎ‬acute exacerbation bronchiectatic ‫اﻏﻠﺐ ﺣﺎﻻت‬

particular Pseudomonas species, antibiotic therapy becomes more challenging and

should be guided by microbiological sensitivities. For Pseudomonas, oral
ciprofloxacin (500–750 mg twice daily) or an intravenous anti-pseudomonal β-
lactam (e.g. piperacillin–tazobactam or ceftazidime) will be required.

Haemoptysis in bronchiectasis often responds to treatment of the underlying

infection, although percutaneous embolisation of the bronchial circulation by an
interventional radiologist may be necessary in the event of massive or repeated
haemoptysis.

Surgical treatment ‫ﻧﺴﻮﯾﮭﺎ ﻓﻘﻂ ﻓﻲ ﺣﺎﻟﺔ ﺟﺰء ﻣﻦ اﻟﺮﺋﺔ ﻣﺘﻀﺮر ﻓﻨﺰﯾﻞ ھﺬا اﻟﺠﺰء‬
‫اﻣﺎ إذا ﻛﻞ اﻟﺮﺋﺔ ﻣﺘﻀﺮرة ﻓﮭﻨﺎ ﻣﺎﺗﻘﺮب ﻋﺎﻟﻤﺮﯾﺾ‬
Excision of bronchiectatic areas is indicated in only a small proportion of cases.
These are usually patients in whom the bronchiectasis is confined to a single lobe
or segment on CT. Unfortunately, many of those in whom medical treatment
proves unsuccessful are also unsuitable for surgery because of either extensive
bilateral bronchiectasis or coexisting severe airflow obstruction. In progressive
forms of bronchiectasis, resection of destroyed areas of lung that are acting as a
reservoir of infection should be considered only as a last resort.

Prognosis

The disease is progressive when associated with ciliary dysfunction or cystic

fibrosis, and eventually causes respiratory failure. In other patients, the prognosis
can be relatively good if physiotherapy is performed regularly and antibiotics are
used aggressively. Longer course +large dose

Prevention

As bronchiectasis commonly starts in childhood following measles, whooping

cough or a primary tuberculous infection, adequate prevention (vaccine) and
treatment of these conditions is essential. Early recognition and treatment of
bronchial obstruction is also important.

Cystic fibrosis

Genetics, pathogenesis and epidemiology

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 Cystic fibrosis (CF) is the most common life-limiting genetic disease in people of
predominantly European descent, with autosomal recessive inheritance, a carrier
rate of 1 in 25, and an incidence of about 1 in 2500 live births in this population,
although worldwide the incidence of CF varies widely.

CF is the result of pathogenic variants affecting a gene on the long arm of

chromosome 7, which codes for a chloride channel known as cystic fibrosis
transmembrane conductance regulator (CFTR); this influences salt and water
movement across epithelial cell membranes.

The genetic defect causes increased sodium and chloride content in sweat and
increased resorption of sodium and water from respiratory epithelium. Relative
dehydration of the airway epithelium is thought to predispose to chronic bacterial
infection, ciliary dysfunction and bronchiectasis. The gene defect also causes
disorders in the gut epithelium, pancreas, liver and reproductive tract.

Clinical features and complications of cystic fibrosis

Respiratory

1- Progressive airway obstruction

2- Infective exacerbations of bronchiectasis

3- Respiratory failure

4- Spontaneous pneumothorax

5- Allergic bronchopulmonary aspergillosis

Gastrointestinal and hepatic

1- Malabsorption and steatorrhoea

2- Distal intestinal obstruction syndrome

3- Increased risk of gastrointestinal malignancy

4- Cystic fibrosis-associated liver disease (CFLD), as biliary cirrhosis.

5- Gallstones

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Others

1- Diabetes (25% of adults)

2- Delayed puberty

3- Male infertility

4- Osteoporosis

5- Arthropathy

Management

Diagnosis

The diagnosis was most commonly made from the clinical picture (bowel
obstruction, failure to thrive, steatorrhoea and/or chest symptoms in a young child),
supported by sweat electrolyte testing and genotyping.

Treatment of CF lung disease

The management of CF lung disease is that of severe bronchiectasis. All patients

with CF who produce sputum should perform chest physiotherapy daily, and more
frequently during exacerbations. Infections with Staph. aureus can often be
managed with oral antibiotics, while intravenous treatment is usually needed for
Pseudomonas infections. Over time patients with CF become chronically infected
with antibiotic-resistant organisms, such as P. aeruginosa, which may require
prolonged treatment with combinations of intravenous antibiotics.

Treatments that reduce chest exacerbations and/or improve lung function in

cystic fibrosis:

1- Mucolytic therapies: Nebulised hypertonic saline, Inhaled mannitol.

2- Inhaled antibiotics: Nebulised tobramycin, Nebulised levofloxacin.

3- Anti-inflammatory therapy: Azithromycin once a day three times weekly

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4- CFTR (cystic fibrosis transmembrane conductance regulator) modulator
therapy: Ivacaftor

Treatment of non-respiratory manifestations of CF

There is a clear link between good nutrition and prognosis in CF. Malabsorption
occurs in 85% of patients due to exocrine pancreatic failure and is treated with oral
pancreatic enzymes and vitamin.

Diabetes eventually develops in over 25% of patients and needs insulin therapy.
Osteoporosis secondary to malabsorption and chronic ill health should be sought
and treated.

References:
Ian D. Penman, Stuart H., et al., editors. Davidson's Principles and Practice of
Medicine. 24th ed., Elsevier Health Sciences, 2022.
Further information

brit-thoracic.org.uk :British Thoracic Society: access to guidelines on a range of respiratory conditions.

ersnet.org :European Respiratory Society: provides information on education and research, and patient
information.

thoracic.org :American Thoracic Society: provides information on education and research, and patient
information.