Systemic Vasculitis - Classification - Large Vessel Vasculitis

Large Vessel Vasculitis

Giant-cell arteritis and Takayasu's arteritis have much in common, but usually affect patients of different ages, with Takayasu's arteritis affecting younger people, and giant-cell arteritis having a later age of onset.

Aortitis can also be considered a large-vessel disease.

Takayasu arteritis. Primarily affects the aorta and its main branches. At least 3 out of 6 criteria yields sensitivity and specificity of 90.5 and 97.8%:

• onset < 40 years affects young and middle -aged women (ages 15–45)
• claudication of extremities
• decreased pulsation of one or both brachial arteries
• at least 10 mmHg systolic difference in both arms
• bruit over one or both carotid arteries or abdominal aorta
• arteriographic narrowing of aorta, its primary branches, or large arteries in upper or lower extremities
• Ocular manifestation
  • visual loss or field defects
  • Retinal hemorrhages
• Neurological abnormalitis
• Treatment: steroids

Giant cell (temporal) arteritis. Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch. At least 3 out of 5 criteria yields sensitivity and specificity of 95 and 91%:

• Age at onset ≥ 50 years
• New onset headache with localized tenderness
• Temporal artery tenderness or decreased pulsation
• Elevated ESR ≥ 50 mm/hour Westergren
• Temporal artery biopsy showing vasculitis with mononuclear cell infiltrate or granulomatous inflammation, usually with multinucleated giant cells