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Takayasu's Arteritis

Summary

  • Chronic, large-vessel vasculitis primarily affecting the aorta and its major branches
  • Characterised by granulomatous inflammation leading to vessel wall thickening, stenosis, and aneurysm formation
  • Imaging findings include vessel wall thickening, luminal narrowing, and collateral vessel formation1

Pathophysiology

  • Exact aetiology unknown, but likely autoimmune in nature
  • Inflammatory process leads to:
    • Granulomatous inflammation of vessel walls
    • Intimal fibrosis and thickening
    • Smooth muscle cell proliferation
    • Eventual stenosis, occlusion, or aneurysm formation
  • T-cell mediated immune response plays a crucial role in pathogenesis

Demographics

  • Predominantly affects young women (80-90% of cases)
  • Typical age of onset: 10-40 years
  • More common in Asian populations, particularly Japan and Southeast Asia
  • Incidence: 1-3 per million per year in North America and Europe

Diagnosis

  • Based on clinical presentation, laboratory findings, and imaging studies
  • American College of Rheumatology criteria (1990) include:
    • Age at disease onset <40 years
    • Claudication of extremities
    • Decreased brachial artery pulse
    • Blood pressure difference >10 mmHg between arms
    • Bruit over subclavian arteries or aorta
    • Arteriogram abnormality
  • Laboratory findings:
    • Elevated erythrocyte sedimentation rate (ESR)
    • Elevated C-reactive protein (CRP)
    • Anaemia of chronic disease

Imaging

  • Conventional angiography:
    • Gold standard for diagnosis
    • Demonstrates luminal changes, stenosis, and occlusions
  • CT angiography (CTA):
    • Vessel wall thickening and enhancement
    • Luminal narrowing or occlusion
    • Collateral vessel formation
  • MR angiography (MRA):
    • Similar findings to CTA
    • Advantages: no radiation exposure, better soft tissue contrast
  • Ultrasound:
    • Useful for carotid and subclavian artery assessment
    • Demonstrates vessel wall thickening and stenosis
  • 18F-FDG PET/CT:
    • Detects active inflammation in vessel walls
    • Useful for monitoring disease activity and treatment response

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  • 15-year-old patient presented with raised inflammatory markers and weight loss.
  • The aortogram showed mural thickening (red arrows) and a 50% narrowing of the infrarenal aorta (blue arrows).
  • Carotid angiography and Doppler ultrasound showed mural thickening (red arrows) and 70% stenosis (blue arrows) of the common carotid arteries.
  • The areas of maximum carotid stenosis were avid on FDG PET.
  • Intracranial arteries and brain perfusion was normal.

Treatment

  • Corticosteroids and immunosuppression/biologics; revascularisation for critical stenosis. Circumferential wall thickening/enhancement of the aorta and its branches in a young woman is the key, with FDG-PET showing activity

Differential diagnosis

Differential Diagnosis Differentiating Feature
Giant Cell Arteritis Predominantly involves temporal, vertebral, and ophthalmic arteries; cranial predilection rather than aortic arch; no subclavian involvement
Fibromuscular Dysplasia "String of beads" alternating narrowing and dilatation on angiography; primarily affects renal and cervical arteries; no wall thickening on MRI
Atherosclerosis Eccentric calcified plaques on CTA; diffuse large vessel involvement without circumferential wall thickening
Syphilitic Aortitis Aortic root dilatation and aneurysm formation; coronary ostial involvement; similar vessel wall thickening to Takayasu
Kawasaki Disease Coronary artery aneurysms on echocardiography/CTA; coronary predilection rather than aortic arch

  1. Somashekar et al. Updates in the diagnosis and management of Takayasu's arteritis. 2023. Postgraduate medicine - Open in new tab. ↩