Table 1

The Chapel Hill nomenclature

Large vessel vasculitis
 Giant cell (temporal) arteritisGranulomatous arteritis of the aorta and its major branches, with a predilection for the extracranial branches of the carotid artery. Often involves the temporal artery. Usually occurs in patients older than 50 and often is associated with polymyalgia rheumatica.
Takayasu arteritisGranulomatous inflammation of the aorta and its major branches. Usually occurs in patients younger than 50.
Medium sized vessel vasculitis
Polyarteritis nodosaNecrotising inflammation of medium sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules.
Kawasaki diseaseArteritis involving large, medium sized, and small arteries, and associated with mucocutaneous lymph node syndrome. Coronary arteries are often involved. Aorta and veins may be involved. Usually occurs in children.
Small vessel vasculitis
Wegener's granulomatosisGranulomatous inflammation involving the respiratory tract, and necrotising vasculitis affecting small to medium sized vessels (that is, capillaries, venules, arterioles, and arteries). Necrotising glomerulonephritis is common.
Churg-Strauss syndromeEosinophil rich and granulomatous inflammation involving the respiratory tract, and necrotising vasculitis affecting small to medium sized vessels, and associated with asthma and eosinophilia.
Microscopic polyangiitisNecrotising vasculitis, with few or no immune deposits, affecting small vessels (that is, capillaries, venules, or arterioles). Necrotising arteritis involving small and medium sized arteries may be present. Necrotising glomerulonephritis is very common. Pulmonary capillaritis often occurs.
Henoch-Schönlein purpuraVasculitis, with IgA dominant immune deposits, affecting small vessels (that is, capillaries, venules, or arterioles). Typically involves skin, gut, and glomeruli, and is associated with arthralgias or arthritis.
Essential cryoglobulinaemic vasculitisVasculitis, with cryoglobulin immune deposits, affecting small vessels (that is, capillaries, venules, or arterioles), and associated with cryoglobulins in serum. Skin and glomeruli are often involved.
Cutaneous leucocytoclastic angiitisIsolated cutaneous leucocytoclastic angiitis without systemic vasculitis or glomerulonephritis.
  • Modified from reference 3.