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Takayasu's arteritis

Introduction

TA affects those under the age of 50. It is rare. There is a granulomatous pan-arteritis of the aorta and its major branches resulting in focal areas of stenosis, occlusion and aneurysmal formation. The disease is often accompanied with systemic symptoms such as arthralgia, fever, weight loss and rashes and headache. When arterial branches occlude the patient presents with stroke. There may be a difference in BP between arms. The ESR and CRP may be elevated. Anti-endothelial antibodies are reported but not specific. The gold standard tests is DSA though diagnosis now being more commonly made with MRI and MRA, CT angiography, PET and high-resolution ultrasound. Certain imaging modalities such as delayed contrast-enhanced MRI sequences and abnormal 18F-FDG-PET uptake are able to detect vascular inflammation in the prestenotic phase.

Diagnostic Criteria

Modified American College of Rheumatology criteria for the diagnosis of Takayasu’s arteritis. 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

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

  • Diagnosis and treatment of cerebral vasculitis, Peter Berlit. Ther Adv Neurol Disord (2010) 3(1) 2942

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