Learning objectives
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Introduction
- It is a rare, chronic large-vessel arteritis
- TA affects those under the age of 50. It is rare.
Pathophysiology
- There is a chronic granulomatous pan-arteritis of the aorta
- Also affects its major branches and pulmonary artery
- Resulting in focal areas of stenosis, occlusion and aneurysmal formation
- When arterial branches occlude the patient presents with stroke.
Clinical
- Systemic symptoms such as arthralgia, fever, weight loss and rashes and headache.
- There may be a difference in BP between arms.
- When arterial branches occlude the patient presents with stroke.
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) |
investigations
- FBC, U&E, ESR and CRP may be elevated.
- Anti-endothelial antibodies are reported but not specific.
- DSA is gold standard but often diagnosis made with MRI and MRA, CTA angiography, PET and high-resolution ultrasound.
- Delayed contrast-enhanced MRI sequences and abnormal 18F-FDG-PET uptake are able to detect vascular inflammation in the pre-stenotic phase.
Management
- About half respond to systemic steroids. Methotrexate and Azathioprine also used. Biological agents have also been used.
- Patients are often given anti-platelets and statins and blood pressure is treated. Intervention is sometimes indicated for severe stenosis.
References and further reading
- Diagnosis and treatment of cerebral vasculitis, Peter Berlit. Ther Adv Neurol Disord (2010) 3(1) 2942
- Takayasu’s disease: a review Cardiol Young 2008; 18: 250–259
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