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

From: Reuma.pt/vasculitis – the Portuguese vasculitis registry

Sub-type of vasculitis N (%)
Large vessel vasculitis
 Takayasu’s arteritis 28 (4)
 Giant cell arteritis 125 (17.8)
 Non-classifiable large vessel vasculitis 10 (1.4)
Medium vessel vasculitis
 Polyarteritis nodosab 26 (3.7)
 Kawasaki disease 1 (0.1)
 Non-classifiable medium vessel vasculitis 2 (0.3)
Small vessel vasculitis
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis
 Microscopic polyangiitis 22 (3.1)
 Granulomatosis with polyangiitis 41 (5.8)
 Eosinophilic granulomatosis with polyangiitis 29 (4.1)
 Non-classifiable ANCA-associated vasculitis 11 (1.6)
Immune complex small vessel vasculitis
 Cryoglobulinaemic vasculitis 20 (2.9)
 IgA vasculitis 16 (2.3)
Variable vessel vasculitis
 Behçet’s disease 298 (42.5)
 Cogan’s syndrome 12 (1.7)
Single-organ vasculitis
 Cutaneous leukocytoclastic angiitis 16 (2.3)
 Cutaneous arteritisb 4 (0.6)
 Primary central nervous system vasculitis 3 (0.4)
 Other 10 (1.4)
Vasculitis associated with systemic disease
 Rheumatoid vasculitis 2 (0.3)
 Other 11 (1.6)
Vasculitis associated with probable etiology
 Hepatitis C virus-associated cryoglobulinaemic vasculitis 5 (0.7)
 Hepatitis B virus-associated vasculitis 1 (0.1)
 Drug-associated immune complex vasculitis 1 (0.1)
 Cancer-associated vasculitis 2 (0.3)
 Other 5 (0.7)
TOTALa 701 (100)
  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