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Table 1 Diagnosis according the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

From: – the Portuguese vasculitis registry

Sub-type of vasculitisN (%)
Large vessel vasculitis
 Takayasu’s arteritis28(4)
 Giant cell arteritis125(17.8)
 Non-classifiable large vessel vasculitis10(1.4)
Medium vessel vasculitis
 Polyarteritis nodosab26(3.7)
 Kawasaki disease1(0.1)
 Non-classifiable medium vessel vasculitis2(0.3)
Small vessel vasculitis
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis
 Microscopic polyangiitis22(3.1)
 Granulomatosis with polyangiitis41(5.8)
 Eosinophilic granulomatosis with polyangiitis29(4.1)
 Non-classifiable ANCA-associated vasculitis11(1.6)
Immune complex small vessel vasculitis
 Cryoglobulinaemic vasculitis20(2.9)
 IgA vasculitis16(2.3)
Variable vessel vasculitis
 Behçet’s disease298(42.5)
 Cogan’s syndrome12(1.7)
Single-organ vasculitis
 Cutaneous leukocytoclastic angiitis16(2.3)
 Cutaneous arteritisb4(0.6)
 Primary central nervous system vasculitis3(0.4)
Vasculitis associated with systemic disease
 Rheumatoid vasculitis2(0.3)
Vasculitis associated with probable etiology
 Hepatitis C virus-associated cryoglobulinaemic vasculitis5(0.7)
 Hepatitis B virus-associated vasculitis1(0.1)
 Drug-associated immune complex vasculitis1(0.1)
 Cancer-associated vasculitis2(0.3)
  1. a The number of diagnoses is higher than the number of patients, given that in 14 cases more than one diagnosis was selected: six patients with cryoglobulinaemic vasculitis and hepatitis C virus-associated cryoglobulinaemic vasculitis (n = 5) or other vasculitis associated with systemic disease (n = 1); three patients with cutaneous leukocytoclastic angiitis and other vasculitis associated with systemic disease (n = 2) or non-classifiable large vessel vasculitis (n = 1); two patients with single-organ vasculitis and granulomatosis with polyangiitis (n = 1) or other vasculitis associated with systemic disease (n = 1); two patients with other vasculitis associated with systemic disease and polyarteritis nodosa (n = 1) or non-classifiable ANCA-associated vasculitis (n = 1); and one patient with Takayasu‘s arteritis and Hepatitis B virus-associated vasculitis
  2. bAll patients with polyarteritis nodosa had systemic involvement of the disease; all patients with cutaneous arteritis had limited forms of polyarteritis nodosa