Page 40 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

16 David S. Younger

Classification and Nosology

The 2012 Revised Chapel Hill Consensus Conference (CHCC) [1] serves as a guide for
the categorization of diverse forms of vasculitis based upon the vessels involved (Table 1).

Large vessel vasculitis (LVV) including giant cell arteritis (GCA) and Takayasu arteritis
(TAK) affects the aorta, its major branches and analogous veins. Medium vessel vasculitis
(MVV) inclusive of polyarteritis nodosa (PAN) and Kawasaki disease (KD) involves main
visceral arteries and veins and initial branches. Small vessel vasculitic (SVV) involvement
affects intraparenchymal arteries, arterioles, capillaries, veins and venules, with a disease
mechanisms related to anti-neutrophil cytoplasmic antibody (ANCA) or immune complexes.

Table 1. Classification of Vasculitides

Large Vessel Vasculitis
Giant cell arteritis
Takayasu arteritis
Medium Vessel Vasculitis
Polyarteritis nodosa
Kawasaki disease
Small Vessel Vasculitis
ANCA-Associated Vasculitis
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Immune-Complex Vasculitis
Cryoglobulinemia
IgA vasculitis (Henoch-Schönlein)
Hypocomplementemic urticarial vasculitis (anti-C1q)
Variable Vessel Vasculitis
Behçet disease
Cogan syndrome
Single Organ Vasculitis
Primary Angiitis of the CNS
Nonsystemic peripheral nerve vasculitis
Idiopathic aortitis (IgG4)
Vasculitis Associated with Systemic Collagen Vascular Disease
Systemic lupus erythematosus
Rheumatoid arthritis vasculitis
Vasculitis Associated with Infection
Acute bacterial meningitis
Tuberculous meningitis
Spirochete disease
Neurosyphilis
Lyme neuroborreliosis
Varicella zoster virus
HIV/AIDS
Adapted from [1].

Complimentary Contributor Copy