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Takayasu arteritis (pulseless disease)

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Anaesthetics and Critical Care

Obstetrics Gynaecology

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Urology

Version Jan 2023

Weight loss, malaise, patient under 40, absent pulse and raised ESR = Takayasu's disease

About

A chronic large vessel vasculitis occurring mainly in women
Affects aorta and its proximal branches

Pathology

Affects the larger arteries usually branches of the aortic arch which basically close off
Causes hypertension, claudication, ischaemia, stroke, TIA, bruits, loss of pulses
The left subclavian and left common carotid are the most commonly narrowed vessels
There may be dilatation of the aortic root and Aortic regurgitation with an EDM

Aetiology

Autoimmune response to an aortic antigen has been suggested
Pathology shows granulomatous arteritis similar to Giant cell arteritis.

Clinical

Malaise, fever, night sweats, fever, arthralgia, tenderness of vessels
Loss of pulses, the difference in BP between arms
Myocardial infarction, Stroke/TIA
Peripheral vascular disease
Aortic regurgitation signs and symptoms

Investigations

? CRP and ? ESR
U&E may be renal impaired
CT/MR/DS Angiography shows coarctation, occlusion and aneurysmal formation.
Negative Autoantibodies (ANA/ANCA/anti-DNA, and antiphospholipid antibodies)

Diagnostic Criteria

Takayasu's arteritis may be diagnosed when at least three of these six criteria are present (sensitivity of 90.5% and a specificity of 97.8%) [Arend et al. 1990]
1	Age at disease onset <50 years
2	Claudication of extremities
3	Decreased brachial artery pulse
4	Blood pressure [systolic] difference > 10mmHg between arms
5	Bruit over subclavian arteries or abdominal aorta
6	Arteriographic narrowing or occlusion of the aorta, its primary branches or large arteries (not due to arteriosclerosis, fibromuscular dysplasia or similar causes)

Management

Takayasu's arteritis is treated with high dose steroids, methotrexate and cyclophosphamide

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