Table 1 Classification of systemic vasculitides according to CHCC nomenclature [3]

Large vessel vasculitis (LVV)

 • Takayasu arteritis (TA)

 • 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’s 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 vasculitis

 • Others