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Takayasu's arteritis


Learning objectives

  • What is Takayasu's arteritis
  • Recognition of Takayasu's arteritis
  • Treatment of Takayasu's arteritis

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

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