Takayasu arteritis !

An inflammation of the aorta and its main branches that mostly affects young women. Narrowed arteries can leave weak or absent arm pulses.

Updated 2026-11-01

Structures involved

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Why it happens

What it is

A chronic granulomatous large-vessel vasculitis of the aorta and its major branches, especially the subclavian, carotid and renal arteries. Inflammation thickens and narrows the arteries, causing stenoses, occlusions and sometimes aneurysms. It is known as «pulseless disease».

Who gets it

Rare, with a strong female predominance; it usually begins before age 40. It is more frequent in Asia and is well described in Latin America, including Mexico and Colombia, where it is among the important causes of renovascular hypertension and absent pulses in young women.

Causes

Autoimmune, cell-mediated inflammation of the arterial wall; cause unknown
Genetic susceptibility (HLA-B*52 association)
A link with prior tuberculosis has been proposed but not proven

Risk factors

Female sex
Age under 40
Asian or Latin American ancestry

Pathophysiology

T cells and macrophages infiltrate the adventitia and media around the vasa vasorum, forming granulomas. The media is destroyed and the intima thickens with fibrosis, producing long smooth stenoses or occlusions; where the wall is weakened, aneurysms form. Narrowing of the subclavian arteries reduces arm pulses and pressure, renal artery stenosis causes hypertension, and aortic root dilation causes aortic regurgitation.

Classification

Early systemic (pre-pulseless) inflammatory phase
Late occlusive (pulseless) phase
Angiographic types by territory (aortic arch and branches, thoracoabdominal aorta, renal arteries, mixed)

Symptoms and signs

Early: fever, night sweats, weight loss, joint and muscle pain
Arm claudication (pain and fatigue on using the arms)
Weak or absent radial or brachial pulses; a difference in systolic pressure of more than 10 mmHg between the arms
Bruits over the subclavian arteries, carotids or abdominal aorta
Dizziness, visual disturbances, syncope or carotid pain (carotidynia)
Hypertension, sometimes severe, from renal artery stenosis

Diagnosis

Clinical: Suspected in a young woman with absent or asymmetric arm pulses, unequal blood pressures between arms, vascular bruits or unexplained hypertension. Blood pressure should be measured in all four limbs; arm readings may underestimate central pressure.
Tests: ESR and CRP are often raised in active disease but may be normal. No specific antibody exists; tuberculosis and syphilis serology help exclude infectious aortitis.
Imaging: Diagnosis rests on imaging: CT or MR angiography shows wall thickening and long tapered stenoses of the aorta and branches; Doppler ultrasound of the carotids and subclavians and PET-CT can show active inflammation. Echocardiography for aortic regurgitation.

Differential diagnosis

Giant cell arteritis (age over 50, temporal artery involvement)
Atherosclerosis
Fibromuscular dysplasia (renal artery stenosis in young women)
Infectious aortitis (tuberculosis, syphilis)
Coarctation of the aorta and other congenital vascular anomalies
Connective tissue disorders with aortic disease (Marfan, Ehlers-Danlos)

Goal of treatment

Control vascular inflammation to stop progression of stenoses, protect brain, heart and kidneys, control blood pressure and restore blood flow where it is critical.

Treatment

Medical: Glucocorticoids are first line to induce remission of active disease.
Medical: Steroid-sparing agents (methotrexate, azathioprine, mycophenolate) or biologics (TNF inhibitors, tocilizumab) for relapsing or refractory disease.
Medical: Blood-pressure control and antiplatelet therapy for ischaemic risk; statins as cardiovascular protection.
Surgical: Angioplasty, stenting or surgical bypass for critical stenoses (renal, carotid, coronary), aortic valve repair for severe regurgitation; done ideally when inflammation is quiet.
Follow-up: Long-term follow-up with clinical examination, inflammatory markers and periodic vascular imaging; nursing teaching to measure blood pressure in the unaffected arm or legs.

Complications

Renovascular hypertension and chronic kidney disease
Stroke and transient ischaemic attacks
Aortic regurgitation and heart failure
Coronary ischaemia and myocardial infarction
Aortic aneurysm
Pregnancy complications (pre-eclampsia, growth restriction)

Prognosis

A chronic relapsing disease; with treatment and revascularisation when needed, long-term survival is good, but organ damage from hypertension, stroke and heart involvement determines the outlook.

Test yourself

Think of the answer, then tap the question to see it.

Who is typically affected by Takayasu arteritis?

Young women, usually under 40 years, more often of Asian or Latin American ancestry.

Why is it called «pulseless disease»?

Stenosis of the subclavian and other aortic branches weakens or abolishes the arm pulses.

Which key bedside finding should prompt suspicion?

A difference of more than 10 mmHg in systolic blood pressure between the arms, often with subclavian bruits.

How is Takayasu arteritis distinguished from giant cell arteritis?

By age: Takayasu begins before 40 and involves the aorta and its branches; giant cell arteritis occurs after 50 and typically involves the temporal arteries.

What is the first-line treatment?

Glucocorticoids, with steroid-sparing agents or biologics for relapsing disease.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: Takayasu arteritis (MedlinePlus (U.S. National Library of Medicine), 2025).

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Representative model, for educational purposes only.

Aortic arch highlighted on the Durgical 3D model