Clinical features and diagnosis of Takayasu

arteritis
Author: Peter A Merkel, MD, MPH
Section Editor: Eric L Matteson, MD, MPH
Deputy Editor: Monica Ramirez Curtis, MD, MPH

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is
complete.

Literature review current through: Oct 2019. | This topic last updated: Jun 17, 2019.

INTRODUCTION

Takayasu arteritis (TAK) is classified as a large-vessel vasculitis because it

primarily affects the aorta and its primary branches. It also shares some histologic
and clinical features with giant cell (temporal) arteritis (GCA), the other major
large-vessel vasculitis. Patients may present initially with constitutional symptoms
but later develop symptoms associated with vascular damage.

The pathogenesis, pathology, clinical manifestations, and diagnosis of TAK will be

reviewed here. The treatment of this disorder is discussed separately (see
"Treatment of Takayasu arteritis"). Overviews of the vasculitides in children and in
adults are also discussed elsewhere. (See "Vasculitis in children: Incidence and
classification" and "Overview of and approach to the vasculitides in adults".)

EPIDEMIOLOGY

Women are affected in 80 to 90 percent of cases, with an age of onset that is

usually between 10 and 40 years [1,2]. It has a worldwide distribution, with the
greatest prevalence in Asia [3-5]. In Japan, it has been estimated that 150 new
cases occur each year [6].

PATHOGENESIS

The pathogenesis of Takayasu arteritis (TAK) is poorly understood. Cell-mediated

mechanisms are thought to be of primary importance and may be similar to those
in giant cell arteritis (GCA) [7]. (See "Pathogenesis of giant cell arteritis".)

Immunohistopathologic examination has shown that the infiltrating cells in aortic

tissue mainly consist of cytotoxic lymphocytes, especially gamma delta T
lymphocytes [8]. These cells may cause vascular injury by releasing large amounts
of the cytolytic protein perforin. Recognition of heat shock protein-65 might
facilitate recognition and adhesion of the infiltrating cells. In another report, the T
cell receptors on the infiltrating T cells had a restricted repertoire, suggesting that
a specific but as yet unidentified antigen in aortic tissue might be targeted [9]. This
change was not seen in atherosclerotic aortic aneurysms.

The inflammation may be localized to a portion of the thoracic or abdominal aorta

and branches, or may involve the entire vessel. Although there is considerable
variability in disease expression (possibly due to geographic differences [10]), the
initial vascular lesions frequently occur in the left middle or proximal subclavian
artery. As the disease progresses, the left common carotid, vertebral,
brachiocephalic, right middle or proximal subclavian artery, right carotid, vertebral
arteries, and aorta may also be affected. The abdominal aorta and pulmonary
arteries are involved in approximately 50 percent of patients [11]. The
inflammatory process within the vessel can lead to narrowing, occlusion, or
dilation of involved portions of the arteries, which causes a wide variety of
symptoms.

CLINICAL FEATURES
Symptoms and signs — The onset of symptoms in Takayasu arteritis (TAK) tends
to be subacute, which often leads to a delay in diagnosis that can range from
months to years, during which time vascular disease may start and progress to
become symptomatic. It is not uncommon for the consequences of the arterial
disease to be the first sign of TAK noticed at presentation. As progression of
narrowing, occlusion, or dilation of arteries occurs, there is resulting pain in arms
or legs (limb claudication) and/or cyanosis, lightheadedness or other symptoms
of reduced blood flow, arterial pain and tenderness, or nonspecific constitutional
symptoms.

The following features can be seen [12-16]:

● Constitutional symptoms – Constitutional symptoms are common in the early

phase of TAK, including weight loss and low-grade fever. Fatigue is very
common.

● Arthralgias – Arthralgias or myalgias occur in about one-half of cases.

Clinically evident synovitis is less common. Articular symptoms can be
transient or continuous over several months or longer.

● Carotidynia – Tenderness of a carotid artery (carotidynia) is observed in 10 to

30 percent of patients at presentation [12].

● Absent or weak peripheral pulse(s) – Absent or diminished peripheral pulses

is most common at the level of the radial arteries and is often asymmetric
[13]. In unusually severe cases, occlusion of the vessels to the extremities
may result in ischemic ulcerations or gangrene; however, such complications
are usually precluded by the development of collateral arterial circulation in
the areas involved by vasculitis, protecting extremities from critical ischemia.
Collateral vessels are evidence of the slow progression of the disease.

● Limb claudication – Limb claudication may be observed. Subclavian artery

involvement is common, and a stenotic lesion proximal to the origin of the
 vertebral artery can lead to neurologic symptoms or syncope related to the
so-called subclavian steal syndrome [17]. In this phenomenon, retrograde flow
through the vertebral artery supplies the subclavian distal to the stenosis and
vasodilation of the arterial bed in the upper limb with exercise compromises
posterior cerebral blood flow (see "Subclavian steal syndrome", section on
'Clinical presentations' and "Subclavian steal syndrome", section on 'Upper
extremity ischemia'). Other claudicatory symptoms are common, including
mild to severe upper- or lower-extremity pain with modest activity, often
limiting patients' functional capacity for activities of daily living, ambulation,
or employment.

● Arterial bruit – In patients with stenoses, bruits are usually audible over the
subclavian arteries, brachial arteries, carotid arteries, and abdominal vessels.
Clinical signs of aortic regurgitation due to dilatation of the ascending aorta
may be present in patients who have this abnormality, and moderate to severe
stenosis can be present even in the absence of a bruit. (See "Examination of
the arterial pulse" and "Clinical manifestations and diagnosis of chronic aortic
regurgitation in adults".)

● Discrepant blood pressure between arms – Reduced blood pressure in one or

both arms is common; a differential of more than 10 mmHg between the
arms is typically present and pressures may be unmeasurable, especially by
automated devices. It is imperative for patients and clinicians to be aware of
such unreliable blood pressure readings to ensure proper pressures are
recorded in limbs not affected by arterial narrowing; this frequently requires
measuring the blood pressure in legs.

● Hypertension – Hypertension develops in more than one-half of cases due to

narrowing of one or both renal arteries, or narrowing and decreased elasticity
of the aorta and branches. Severe (malignant) hypertension may occur.
However, narrowing or occlusion of the arteries in the arms may make it
 difficult to assess the blood pressure. In these cases, the blood pressure may
be measured using a wide cuff on an uninvolved thigh, or by direct
measurements of the proximal aorta via arterial catheterization. Because TAK
often affects young people, modest elevations in blood pressure are often
overlooked. (See "Blood pressure measurement in the diagnosis and
management of hypertension in adults".)

● Angina – Angina pectoris occurs due to coronary artery ostial narrowing from
aortitis or coronary arteritis. Myocardial infarction and death may occur.

● Gastrointestinal symptoms – Abdominal pain, particularly post-prandial pain,

diarrhea, and gastrointestinal hemorrhage may result from mesenteric artery
ischemia [16].

● Skin lesions – Skin lesions resembling erythema nodosum or pyoderma

gangrenosum are found over the legs in a minority of cases.

● Respiratory symptoms – The pulmonary arteries are involved pathologically

in up to 50 percent of cases; however, symptoms related to pulmonary
arteritis are less common [15]. Pulmonary manifestations include chest pain,
dyspnea, hemoptysis, and pulmonary hypertension [16]. Dyspnea may also be
due to angina or heart failure resulting from aortic dilation, aortic
regurgitation, or malignant hypertension.

● Neurologic symptoms – Involvement of the carotid and vertebral arteries

causes decreased cerebral blood flow, leading to lightheadedness, vertigo,
syncope, orthostasis, headaches, convulsions, and strokes. Visual impairment
is a late manifestation of severe disease and is due to arterial insufficiency
[14].

Physical examination — Several aspects of the physical examination merit

particular attention whenever a patient TAK is seen in clinical practice.
Measurement of blood pressure should be done in all four extremities to evaluate
for arterial stenoses and accurately measure the true central arterial pressure.
Many patients with TAK will have partial or complete occlusion of one or both
subclavian, axillary, or brachial arteries, or the brachiocephalic artery, leading to
falsely low pressure readings in the ipsilateral arm. Similarly, femoral or more
distal arterial stenoses will falsely lower leg blood pressures and stenosis of the
aorta may lead to bilateral low blood pressure readings. It is important that
clinicians, nurses, and other clinical staff enter all four extremity blood pressure
readings into a patient's medical records to best allow for determination of which
readings are reliable and to gain insight into the patient's vascular anatomy.

Bruits should be listened for over the bilateral carotid, subclavian, axillary, renal,
and femoral arteries, as well as the abdominal aorta. Cardiac auscultation may
reveal signs of aortic valvular disease, pulmonary hypertension, or heart failure.
Pulses should be felt for and evaluated (full, reduced, absent) at bilateral temporal,
carotid, brachial, femoral, and dorsal pedal arteries, and any arterial tenderness
should also be noted. Signs of limb ischemia should be sought. Availability of a
device using Doppler technology can enhance the vascular examination in patients
with TAK. Physical examination may reveal findings suggestive of vascular
disease. Many of the abnormal exam findings above have been shown to be fairly
specific, although not highly sensitive, for identification of arterial lesions
subsequently confirmed by imaging tests [18].

Laboratory findings — Laboratory abnormalities in patients with TAK are

nonspecific and generally reflect an inflammatory process [12]. Acute phase
reactants such as erythrocyte sedimentation rate (ESR) and C-reactive protein
(CRP) may be elevated; however, these tests do not reliably reflect disease activity
and can be normal in the setting of active disease [16].

Other abnormalities that may be observed in the complete blood count include a
normochromic normocytic anemia suggestive of the anemia of chronic disease as
well as a leukocytosis and/or thrombocytosis.
DIAGNOSIS

Our approach — In most cases, a clinical diagnosis of Takayasu arteritis (TAK) can
be made in a patient with both suggestive clinical findings (eg, constitutional
symptoms, hypertension, diminished or absent pulses, and/or arterial bruits) and
imaging showing narrowing of the aorta and/or its primary branches. (See
'Symptoms and signs' above and 'Imaging' below.)

Occasionally, the diagnosis of TAK is made incidentally either in patients with

imaging consistent with vasculitis obtained for other clinical indications (eg,
"aortitis" suspected on an abdominal computed tomography [CT] obtained to
evaluate for possible malignancy or incidentally discovered aortic aneurysm) or
when vasculitis is found on histologic examination of surgically removed
segments of arteries. In such circumstances, we recommend examining other
regions of the aorta and its primary branches with either magnetic resonance
angiography (MRA) or CT angiography (CTA) and considering the possibility of a
diagnosis of another form of large-vessel vasculitis.

There are no diagnostic laboratory tests for TAK. Testing for acute phase
reactants such as the erythrocyte sedimentation rate (ESR) and C-reactive protein
(CRP) may provide additional support for the presence of a systemic inflammatory
process; however, normal values of ESR or CRP should not markedly deter making
the diagnosis of TAK.

Imaging — Imaging studies are essential for establishing the diagnosis of TAK

and for determining the extent of vascular involvement. Patients with suspected
TAK should undergo imaging of the arterial tree by MRA or CTA to evaluate the
arterial lumen. The diagnostic and physiologic importance of arterial wall
enhancement on MR or CT imaging remain uncertain. In general, we favor using
MRA to evaluate for TAK since it avoids the radiation exposure and risks of
iodinated contrast of CTA; similarly, if periodic repeat studies are anticipated, MRA
is again the preferred choice.

Imaging of the arterial tree of the chest, abdomen, head and neck, or other areas
by MRA or CTA demonstrates smoothly tapered luminal narrowing or occlusion
(image 1) that is sometimes accompanied by thickening of the wall of the vessel
(image 2A-B) [19-24]. Color Doppler ultrasound examination of the common
carotid and proximal subclavian arteries may show vessel wall thickening and
luminal narrowing, especially if bruits or diminished pulses are found on
examination, and may provide complementary information to MRA/CTA about
hemodynamics. However, ultrasound examination cannot reach vessels in deeper
areas, and multiple procedures and more time are necessary to cover the same
regions seen by a single MR or CT study.

While conventional arteriography generally provides clear outlines of the lumen of

involved arteries (image 3), it does not allow arterial wall thickening to be
assessed (the importance of which is not fully clear) and is an invasive test
associated with some risks. Therefore, if a therapeutic intervention (eg, stenting
for revascularization) is not anticipated, a less invasive imaging technique is
preferred. (See "Treatment of Takayasu arteritis", section on 'Revascularization'.)

Catheter-based angiography allows for the measurement of core blood pressure,

and this may be needed when four-limb arterial stenosis prevents accurate blood
pressure assessments; documenting aortic pressures should always be part of
this procedure. Furthermore, cardiac catheterization may be a critically important
form of angiography to perform when cardiac ischemia is suspected. When
selected site angiography is planned, it may be quite appropriate to extend the
scope of the study to include more/all of the aorta and primary branches since the
initial risk of arterial puncture was already planned; time, costs, and the risks of
using additional contrast dye must be considered.
Positron emission tomography (PET), often in combination with CT (PET-CT) or
MR (PET-MR) is an increasingly utilized test to evaluate for possible large-vessel
vasculitis. The finding of "hot" segments (ie, those with increased standardized
uptake values [SUVs]) in the right clinical setting may be quite suggestive of large-
vessel vasculitis. However, the exact role for PET in diagnosis of TAK is not clear
and the use of PET to measure disease activity in large-vessel vasculitis remains
under study [25].

Histopathology — The diagnosis of TAK is seldom made histologically since

biopsy of the large arteries is obviously impractical. However, occasionally arterial
tissue may become available after a revascularization procedure or aneurysm
repair. Such tissue samples should be sought whenever reasonably feasible if it
will help either establish a diagnosis or evaluate the state of the disease (eg,
active inflammation versus inactive scar) and lead to change in therapy.

Active inflammation is indicated by the presence of mononuclear cells,

predominantly lymphocytes, histiocytes, macrophages, and plasma cells [26].
Giant cells and granulomatous inflammation are typically found in the media [27].
Destruction of the elastic lamina and the muscular media can lead to aneurysmal
dilation of the affected vessel. Alternatively, progressive inflammation and dense
scarring may proceed from the adventitia leading to a compromise of the vascular
lumen. Intimal proliferation may also contribute to the development of stenotic
arterial lesions. If active inflammation abates, dense scar tissue remains as an
indication of prior vasculitis.

Nomenclature and classification criteria — The disease names and definitions of

the vasculitides continue to evolve as our understanding of the pathogenesis
advances. The international Chapel Hill Consensus Conference (CHCC) has
developed one of the most widely used nomenclature systems which specifies the
names and definitions for most forms of vasculitis [28,29]. The CHCC
nomenclature system has changed over the past few decades, and definitions that
were put forth by the CHCC in 1994 have since been revised in the 2012 CHCC
(table 1 and table 2). (See "Overview of and approach to the vasculitides in adults",
section on 'Nomenclature'.)

Classification criteria have been developed for TAK as a means of categorizing

patents for research studies. The American College of Rheumatology (ACR)
classification criteria were developed to help distinguish one form of vasculitis
from another, but they are limited in terms of their use in clinical practice [2,30,31].

● Age at disease onset ≤40 years

● Claudication of the extremities

● Decreased pulsation of one or both brachial arteries

● Difference of at least 10 mmHg in systolic blood pressure between the arms

● Bruit over one or both subclavian arteries or the abdominal aorta

● Arteriographic narrowing or occlusion of the entire aorta, its primary

branches, or large arteries in the proximal upper or lower extremities, not due
to arteriosclerosis, fibromuscular dysplasia, or other causes

Patients are said to have TAK if at least three of the six criteria are present.

Although the ACR criteria and the CHCC nomenclature system have been widely
used by clinical researchers and clinicians to help diagnose patients, accurate
diagnostic criteria have yet to be developed. With an increased understanding of
the pathophysiology of vasculitis and improved laboratory testing, the ACR and the
European League Against Rheumatism (EULAR) are in the process of making an
international effort to develop revised classification criteria and diagnostic criteria
[32,33].

DIFFERENTIAL DIAGNOSIS
The differential diagnosis of Takayasu arteritis (TAK) includes atherosclerotic,
inflammatory, infectious, and hereditary diseases that affect the large arteries.

● Giant cell arteritis – Perhaps the most difficult distinction is between TAK and
giant cell arteritis (GCA). Both conditions involve the aorta and its major
branches and are indistinguishable histopathologically. Distinction between
the two disorders can usually be made based upon the age of the patient and
the distribution of lesions (table 3) [34,35], although such a dichotomy is
strongly driven the by the somewhat arbitrary age-based criteria.

However, with the increasing recognition of overlapping clinical features of

these two conditions, and especially that GCA may involve the aorta and its
major branches in at least 30 percent of patients, the differentiation between
TAK and GCA has become more difficult [36,37]. (See "Diagnosis of giant cell
arteritis" and "Clinical manifestations of giant cell arteritis".)

● Other forms of large-vessel vasculitis/aortitis – There are several other

diseases associated with aortitis that can present with clinical and
radiographic features identical to TAK, including Cogan's syndrome, relapsing
polychondritis, and spondyloarthropathies. However, in most of these
situations, the finding of other clinical features pathognomonic for the
specific diagnosis can differentiate among these types of aortitis. (See
"Cogan's syndrome" and "Clinical manifestations of relapsing polychondritis"
and "Overview of the clinical manifestations and classification of
spondyloarthritis".)

● Behçet syndrome – Arterial involvement in Behçet syndrome can lead to

dilatations and aneurysms of the medium- and large-sized arteries. However,
patients with Behçet are likely to have other clinical features such as oral
and/or genital ulcerations, ocular disease, and arthritis. (See "Clinical
manifestations and diagnosis of Behçet syndrome".)
● IgG4-related disease – IgG4-related disease has been recognized as a rare
cause of noninfectious aortitis. Histologic evidence of lymphoplasmocytes
and storiform fibrosis as well as non-arterial manifestations of disease can
differentiate IgG4-related disease from TAK. (See "Pathogenesis and clinical
manifestations of IgG4-related disease", section on 'Aortitis and periaortitis'.)

● Infectious aortitis – Similar to TAK, patients with infectious aortitis may

present with nonspecific symptoms such as fever and elevated acute phase
reactants. However, patients with TAK will have negative blood cultures.
Infections of the aorta usually lead to aneurysm formation. Mycobacterial
infection of the aorta can present with a more chronic process that bacterial
infections. On computed tomography angiography (CTA), patients with
infectious aneurysms may have a perivascular fluid collection or intramural
air, whereas inflammatory aneurysms often have findings suggestive of
periaortic fibrosis and adhesions of adjacent structures. (See "Overview of
infected (mycotic) arterial aneurysm", section on 'Infected versus
inflammatory aneurysm'.)

● Genetic causes of aortic aneurysms – Genetic defects that lead to

abnormalities in connective tissue metabolism can predispose patients to
thoracic aortic aneurysm and dissection. Examples include Marfan syndrome,
vascular Ehlers-Danlos syndrome, Loeys-Dietz syndrome, and Turner
syndrome. Patients with these conditions generally do not have systemic
symptoms as do patients with TAK. These conditions are associated with a
specific genetic abnormality and other typical clinical features. (See
"Epidemiology, risk factors, pathogenesis, and natural history of thoracic
aortic aneurysm", section on 'Syndromic connective tissue disorders' and
"Epidemiology, risk factors, pathogenesis, and natural history of thoracic
aortic aneurysm", section on 'Turner syndrome'.)
 ● Fibromuscular dysplasia – Fibromuscular dysplasia must be considered when
large arterial stenosis are seen. However, this syndrome often has
characteristic radiographic findings, is usually more focal in its involvement,
and is not associated with the systemic symptoms of TAK. (See "Clinical
manifestations and diagnosis of fibromuscular dysplasia".)

● Atherosclerosis – It is likely that atherosclerosis is the cause of more lesions

of the aorta and its major branches than all forms of inflammatory disease
combined. Differentiating atherosclerosis from vasculitis may be easier in
younger people but can be a challenge in many patients. Longer, smoother,
non-calcified lesions are more characteristic of non-atherosclerotic disease.
However, atherosclerosis can be associated with some degree of
inflammation and increased signal on positron emission tomography (PET),
and luminal characteristics are not fully reliable when evaluating lesions.
Furthermore, patients with large-vessel vasculitis can also develop
atherosclerosis. Evaluation of all patients with TAK for risk factors and
evidence of atherosclerosis is appropriate. (See "Overview of established risk
factors for cardiovascular disease".)

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries

and regions around the world are provided separately. (See "Society guideline
links: Takayasu arteritis".)

INFORMATION FOR PATIENTS

UpToDate offers two types of patient education materials, "The Basics" and
"Beyond the Basics." The Basics patient education pieces are written in plain
language, at the 5th to 6th grade reading level, and they answer the four or five key
questions a patient might have about a given condition. These articles are best for
patients who want a general overview and who prefer short, easy-to-read
materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th
grade reading level and are best for patients who want in-depth information and
are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We
encourage you to print or e-mail these topics to your patients. (You can also locate
patient education articles on a variety of subjects by searching on "patient info"
and the keyword(s) of interest.)

● Beyond the Basics topics (see "Patient education: Vasculitis (Beyond the
Basics)")

SUMMARY AND RECOMMENDATIONS

● Takayasu arteritis (TAK) is an uncommon chronic vasculitis of unknown
etiology, which primarily affects the aorta and its primary branches. Women
are affected in 80 to 90 percent of cases, with an age of onset that is usually
between 10 and 40 years. It has a worldwide distribution, with the greatest
prevalence in Asia. (See 'Introduction' above and 'Epidemiology' above.)

● The pathogenesis of TAK is poorly understood. The inflammation may be

localized to a portion of the thoracic or abdominal aorta and branches, or may
involve the entire vessel. Although there is considerable variability in disease
expression, the initial vascular lesions frequently occur in the left middle or
proximal subclavian artery. As the disease progresses, the left common
carotid, vertebral, brachiocephalic, right middle or proximal subclavian artery,
right carotid, vertebral arteries, and aorta may also be affected. The
abdominal aorta and pulmonary arteries are involved in approximately 50
percent of patients. The inflammatory process within the vessel can lead to
 narrowing, occlusion, or dilation of involved portions of the arteries in which
causes a wide variety of symptoms. (See 'Pathogenesis' above.)

● The onset of symptoms in TAK tends to be subacute, which often leads to a

delay in diagnosis that can range from months to years, during which time
vascular disease may start and progress. It is not uncommon for the
consequences of the arterial disease to be the first sign of TAK noticed at
presentation. As progression of narrowing, occlusion, or dilation of arteries
occurs, there is resulting pain in arms or legs (limb claudication) and/or
cyanosis, lightheadedness or other symptoms of reduced blood flow, arterial
pain and tenderness, or nonspecific constitutional symptoms. (See
'Symptoms and signs' above.)

● The physical examination of a patients with TAK should particularly focus on

accurate measurements of blood pressure, palpation of pulses, identification
of bruits, and careful cardiac auscultation. (See 'Physical examination' above.)

● The diagnosis of TAK should be suspected in a patient who has constitutional

symptoms, hypertension, diminished or absent pulses, and/or arterial bruits.
In most cases, the diagnosis is based upon suggestive clinical features and
specific imaging findings of the aorta and/or its branches. Additionally, TAK or
other forms of large-vessel vasculitis must be considered either in patients
incidentally found to have findings suspicious for vasculitis on imaging
obtained for other clinical indications or when vasculitis is found on histologic
examination of surgically removed segments of arteries. (See 'Our approach'
above.)

● There are no diagnostic laboratory tests for TAK. Testing for acute phase
reactants such as the erythrocyte sedimentation rate (ESR) and C-reactive
protein (CRP) may provide additional support for the presence of a systemic
inflammatory process; however, normal values of ESR or CRP should not
 markedly deter making the diagnosis of TAK. (See 'Our approach' above and
'Laboratory findings' above.)

● Imaging studies are essential for establishing the diagnosis of TAK and for
determining the extent of vascular involvement. Patients with suspected TAK
should undergo imaging of the arterial tree by magnetic resonance
angiography (MRA) or computed tomography angiography (CTA) to evaluate
the arterial lumen. In general, we favor using MRA to evaluate for TAK, since it
avoids the radiation exposure and risks of iodinated contrast of CTA; similarly,
if periodic repeat studies are anticipated, MRA is again the preferred choice.
Imaging of the arterial tree of the chest, abdomen, head and neck, or other
areas by MRA or CTA demonstrates smoothly tapered luminal narrowing or
occlusion (image 1) that is sometimes accompanied by thickening of the wall
of the vessel (image 2A-B). (See 'Imaging' above.)

● Conditions that should be considered in the differential diagnosis of TAK

include giant cell (temporal) arteritis (GCA), IgG4-related disease, Behçet
syndrome, infectious aortitis, fibromuscular dysplasia, atherosclerosis,
genetic causes of aneurysms such as Ehlers-Danlos syndrome, and other
diseases which can feature large-vessel vasculitis/aortitis such as Cogan's
syndrome, relapsing polychondritis, and spondyloarthropathies. (See
'Differential diagnosis' above.)

ACKNOWLEDGMENT

The editorial staff at UpToDate would like to acknowledge Gene Hunder, MD, who
contributed to an earlier version of this topic review.

REFERENCES
 1. Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, et al. Takayasu's arteritis.
Clinical study of 107 cases. Am Heart J 1977; 93:94.

2. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology
1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;
33:1129.

3. Dabague J, Reyes PA. Takayasu arteritis in Mexico: a 38-year clinical

perspective through literature review. Int J Cardiol 1996; 54 Suppl:S103.

4. Hall S, Barr W, Lie JT, et al. Takayasu arteritis. A study of 32 North American
patients. Medicine (Baltimore) 1985; 64:89.

5. Ishikawa K. Natural history and classification of occlusive thromboaortopathy

(Takayasu's disease). Circulation 1978; 57:27.

6. Koide K. Takayasu arteritis in Japan. Heart Vessels Suppl 1992; 7:48.

7. Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med
2003; 349:160.

8. Seko Y, Minota S, Kawasaki A, et al. Perforin-secreting killer cell infiltration and

expression of a 65-kD heat-shock protein in aortic tissue of patients with
Takayasu's arteritis. J Clin Invest 1994; 93:750.

9. Seko Y, Sato O, Takagi A, et al. Restricted usage of T-cell receptor Valpha-

Vbeta genes in infiltrating cells in aortic tissue of patients with Takayasu's
arteritis. Circulation 1996; 93:1788.

10. Cid MC, Font C, Coll-Vinent B, Grau JM. Large vessel vasculitides. Curr Opin
Rheumatol 1998; 10:18.

11. Yamada I, Shibuya H, Matsubara O, et al. Pulmonary artery disease in

Takayasu's arteritis: angiographic findings. AJR Am J Roentgenol 1992;
159:263.
12. Mason JC. Takayasu arteritis--advances in diagnosis and management. Nat
Rev Rheumatol 2010; 6:406.

13. Serra R, Butrico L, Fugetto F, et al. Updates in Pathophysiology, Diagnosis and

Management of Takayasu Arteritis. Ann Vasc Surg 2016; 35:210.

14. Rodríguez-Pla A, de Miguel G, López-Contreras J, et al. Bilateral blindness in

Takayasu's disease. Scand J Rheumatol 1996; 25:394.

15. Nakabayashi K, Kurata N, Nangi N, et al. Pulmonary artery involvement as first

manifestation in three cases of Takayasu arteritis. Int J Cardiol 1996; 54
Suppl:S177.

16. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med
1994; 120:919.

17. Yoneda S, Nukada T, Tada K, et al. Subclavian steal in Takayasu's arteritis. A

hemodynamic study by means of ultrasonic Doppler flowmetry. Stroke 1977;
8:264.

18. Grayson PC, Tomasson G, Cuthbertson D, et al. Association of vascular

physical examination findings and arteriographic lesions in large vessel
vasculitis. J Rheumatol 2012; 39:303.

19. Hata A, Numano F. Magnetic resonance imaging of vascular changes in

Takayasu arteritis. Int J Cardiol 1995; 52:45.

20. Yamada I, Numano F, Suzuki S. Takayasu arteritis: evaluation with MR

imaging. Radiology 1993; 188:89.

21. Yamada I, Nakagawa T, Himeno Y, et al. Takayasu arteritis: evaluation of the

thoracic aorta with CT angiography. Radiology 1998; 209:103.

22. Paul JF, Hernigou A, Lefebvre C, et al. Electron beam CT features of the
pulmonary artery in Takayasu's arteritis. AJR Am J Roentgenol 1999; 173:89.
23. Kissin EY, Merkel PA. Diagnostic imaging in Takayasu arteritis. Curr Opin
Rheumatol 2004; 16:31.

24. Keenan NG, Mason JC, Maceira A, et al. Integrated cardiac and vascular
assessment in Takayasu arteritis by cardiovascular magnetic resonance.
Arthritis Rheum 2009; 60:3501.

25. Grayson PC, Alehashemi S, Bagheri AA, et al. 18 F-Fluorodeoxyglucose-

Positron Emission Tomography As an Imaging Biomarker in a Prospective,
Longitudinal Cohort of Patients With Large Vessel Vasculitis. Arthritis
Rheumatol 2018; 70:439.

26. Fassbender HG. Pathology and pathobiology of rheumatic diseases, Second E

dition, Springer-Verlag, Berlin 2002. p.304.

27. Nasu T. Takayasu's truncoarteritis. Pulseless disease or aortitis syndrome.

Acta Pathol Jpn 1982; 32 Suppl 1:117.

28. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill
Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;
65:1.

29. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic

vasculitides. Proposal of an international consensus conference. Arthritis
Rheum 1994; 37:187.

30. Ishikawa K. Diagnostic approach and proposed criteria for the clinical
diagnosis of Takayasu's arteriopathy. J Am Coll Cardiol 1988; 12:964.

31. Sharma BK, Jain S, Suri S, Numano F. Diagnostic criteria for Takayasu
arteritis. Int J Cardiol 1996; 54 Suppl:S141.

32. Craven A, Robson J, Ponte C, et al. ACR/EULAR-endorsed study to develop

Diagnostic and Classification Criteria for Vasculitis (DCVAS). Clin Exp Nephrol
2013; 17:619.
33. Luqmani RA, Suppiah R, Grayson PC, et al. Nomenclature and classification of
vasculitis - update on the ACR/EULAR diagnosis and classification of
vasculitis study (DCVAS). Clin Exp Immunol 2011; 164 Suppl 1:11.

34. Hunder GG. Giant cell arteritis and polymyalgia rheumatica. Med Clin North
Am 1997; 81:195.

35. Michel BA, Arend WP, Hunder GG. Clinical differentiation between giant cell
(temporal) arteritis and Takayasu's arteritis. J Rheumatol 1996; 23:106.

36. Grayson PC, Maksimowicz-McKinnon K, Clark TM, et al. Distribution of arterial

lesions in Takayasu's arteritis and giant cell arteritis. Ann Rheum Dis 2012;
71:1329.

37. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Takayasu arteritis and

giant cell arteritis: a spectrum within the same disease? Medicine (Baltimore)
2009; 88:221.

Topic 8238 Version 18.0

© 2019 UpToDate, Inc. All rights reserved.