Reuma.pt/vasculitis – the Portuguese vasculitis registry

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

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 nodosa^b	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 arteritis^b	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)
TOTAL^a	701	(100)

^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
^bAll patients with polyarteritis nodosa had systemic involvement of the disease; all patients with cutaneous arteritis had limited forms of polyarteritis nodosa

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