Tag Archives: Rheumatology

Takayasu Arteritis

Background:
  • Chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches
  • Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation
  • Symptoms reflect end organ ischaemia. More acute inflammation can destroy the arterial media and lead to aneurysm formation
Manifestations:
  • Range from asymptomatic disease found as a result of impalpable pulses or bruits, to catastrophic neurological impairment
  • Non-specific features include fever, night sweats, malaise, weight loss, arthralgia, myalgia, and mild anemia
ACR Diagnostic Criteria – 3 of 6 should be positive (sensitivity 90.5%, specificity 97.8%)
  • Age at onset < 40 years
  • Claudication of the extremities
  • Decreased brachial artery pressure
  • BP difference > 10mmHg between arms
  • Bruit over subclavian arteries or abdominal aorta
  • Arteriogram abnormality: narrowing or occlusion of entire aorta, its branches, or large arteries in the extremities. Usually focal or segmental. Not due to arteriosclerosis or FMD.
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Treatment:
  • Medical
    • Mainstay: Glucocorticoids – effectively suppress the systemic symptoms and usually arrest disease progression. Arterial stenosis may reverse, and ischemic symptoms may improve in early cases.
    • Refractory disease:azathioprine, mycophenolate, methotrexate, tocilizumab, or leflunomide and reserve cyclophosphamide for those who have continued disease activity despite those medications
  • Surgical
    • Irreversible arterial stenosis/significant ischemic symptoms: angioplasty v bypass