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Last updated July 8, 1999
Nomenclature of Vasculitis
Names and Definitions of Vasculitis Adopted by the Chapel Hill Consensus
Conference on the Nomenclature of Systemic Vasculitis.
Click here to see the slideshow.
LARGE VESSEL VASCULITIS1
Giant cell (temporal)
arteritis
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Granulomatous 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.
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Takayasu arteritis |
Granulomatous inflammation of the aorta and its major branches.
Usually occurs in patients younger than 50.
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MEDIUM-SIZED VESSEL VASCULITIS1
Polyarteritis nodosa2
(classic polyarteritis
nodosa)
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Necrotizing inflammation of medium-sized or small arteries without
glomerulonephritis or vasculitis in arterioles, capillaries or venules.
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Kawasaki disease
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Arteritis 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.
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SMALL VESSEL VASCULITIS1
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Wegener's granulomatosis3
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Granulomatous inflammation involving the respiratory tract, and necrotizing
vasculitis affecting small to medium-sized vessels, e.g. capillaries,
venules, arterioles, and arteries.
Necrotizing glomerulonephritis is common.
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Churg-Strauss syndrome3
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Eosinophil-rich and granulomatous inflammation involving the respiratory
tract and necrotizing vasculitis affecting small to medium-sized vessels,
and associated with asthma and blood eosinophilia.
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Microscopic polyangiitis2,3
(microscopic polyarteritis)
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Necrotizing vasculitis with few or no immune deposits affecting small
vessels, i.e. capillaries, venules, or arterioles.
Necrotizing arteritis involving small and medium-sized arteries may be
present. Necrotizing glomerulonephritis is very common.
Pulmonary capillaritis often occurs.
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Henoch-Schönlein purpura
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Vasculitis with IgA-dominant immune deposits affecting small vessels, i.e.
capillaries, venules, or arterioles.
Typically involves skin, gut and glomeruli, and is associated with
arthralgias or arthritis.
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Essential cryoglobulinemic
vasculitis
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Vasculitis with cryoglobulin immune deposits affecting small vessels,
i.e. capillaries, venules, or arterioles, and associated with cryoglobulins
in serum.
Skin and glomeruli are often involved.
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Cutaneous leukocytoclastic
angiitis
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Isolated cutaneous leukocytoclastic angiitis without systemic vasculitis or
glomerulonephritis.
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1 Large artery refers to the aorta and the largest branches directed
toward major body regions (e.g., to the extremities and the head and neck);
medium-sized artery refers to the main visceral arteries (e.g. renal,
hepatic, coronary and mesenteric arteries), and small artery refers to the
distal arterial radicals that connect with arterioles. Note that some small
and large vessel vasculitides may involve medium-sized arteries; but large
and medium-sized vessel vasculitides do not involve vessels smaller than
arteries.
2 Preferred term.
3 Strongly associated with antineutrophil cytoplasmic autoantibodies
(ANCA).
Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic
vasculitides: proposal of an international consensus committee.
Arthritis & Rheum. 1994, 37:187-192.
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