Page 149 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Nomenclature and Pathologic Features of Vasculitides 125

In addition to the vascular distribution, LVV is characterized pathologically by a
predominance of monocytes, macrophages, sometimes including multinucleated giant cells
and lymphocytes even in the acute phase of vascular inflammation [3-5].

Table 1. Names for vasculitides adopted by the 2012 International Chapel Hill
Consensus Conference on the Nomenclature of Vasculitides

Large vessel vasculitis (LVV)
Takayasu arteritis (TAK)
Giant cell arteritis (GCA)
Medium vessel vasculitis (MVV)
Polyarteritis nodosa (PAN)
Kawasaki disease (KD)
Small vessel vasculitis (SVV)
Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV)
Microscopic polyangiitis (MPA)
Granulomatosis with polyangiitis (Wegener?s) (GPA)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)
Immune complex SVV
Anti-glomerular basement membrane (anti-GBM) disease
Cryoglobulinemic vasculitis (CV)
IgA vasculitis (Henoch-Schönlein) (IgAV)
Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)
Variable vessel vasculitis (VVV)
Behçet disease (BD)
Cogan?s syndrome (CS)
Single-Organ vasculitis (SOV)
Cutaneous leukocytoclastic angiitis
Cutaneous arteritis
Primary central nervous system vasculitis Isolated aortitis
Others
Vasculitis associated with systemic disease
Lupus vasculitis
Rheumatoid vasculitis
Sarcoid vasculitis
Others
Vasculitis associated with probable etiology
Hepatitis C virus–associated cryoglobulinemic vasculitis
Hepatitis B virus–associated vasculitis
Syphilis-associated aortitis
Drug-associated immune complex vasculitis
Drug-associated ANCA-associated vasculitis
Cancer-associated (paraneoplastic) vasculitis
Others

In addition, overt fibrinoid necrosis of the vessel wall is absent or a minor feature of the
lesion, whereas in the acute phase of MVV and SVV there often is a predominance of

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