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 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) |
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