February 10, 2013

Updated Nomenclature of Vasculitides

A sagittal-curved-reformatted CT image of the aorta of a 31-year-old man demonstrate extensively calcified intima and focal narrowing of the mid/distal thoracic aorta, consistent with Takayasu arteritis.

Very recently, the international consensus conference addressed the revision of the nomenclature of systemic vasculitides as follows:

  • LARGE-vessel vasculitis: Takayasu arteritis and giant cell arteritis
  • MEDIUM-vessel vasculitis: polyarteritis nodosa, Kawasaki disease
  • SMALL-vessel vasculitis: ANCA-associated vasculitis (microscopic polyangiitis, Wegener, Churg-Strauss), immune complex vasculitis 
  • VARIABLE-vessel vasculitis: Behcet disease, Cogan syndrome
  • SINGLE-ORGAN vascuiltis: cutaneous leukocytoclastic angiitis, primary CNS vasculitis, and others
  • Vasculitis associated with systemic diseases such as lupus, rheumatoid arthritis and sarcoid
  • Vasculitis associated with probable etiology (e.g., associated with viral hepatitis, drugs) 
Categorization by vessel size reflects the arteries those are predominantly affected. Vasculitis in each category can affect any size artery. 

Jennette JC, et al. 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013;65:1-11.

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