Background Granulomatosis with polyangiitis is a systemic necrotizing vasculitis of unknown origin with distinct racial, ethnic risk, and characteristics.

Objectives Little is known about the features and prognostic factors in Korean patients with granulomatosis with polyangiitis. We retrospectively studied the risk factors predictive of long term survival in systemic granulomatosis with polyangiitis according to the ACR classification criteria¹.

Methods Twenty four cases of systemic granulomatosis with polyangiitis, diagnosed at a single tertiary hospital from 1996 to 2011, were identified. Univariate and Kaplan Meier analyses of survival were performed using the clinical, laboratory, and treatment parameters.

Results The mean age was 48 ± 18 (18-75) years and C-antineutrophil cytoplasm antibodies (ANCA) and/or PR3 antibody were present in 15 of 24 patients (62.5%). Main clinical features were constitutional symptoms (96%) followed by lung (83%), ENT (83%), kidney (46%), nervous system (33%), skin (33%), and GI (21%) involvement. Two prognostic scoring systems, the Five Factor Score (FFS) and Birmingham Vasculitis Activity Score (BVAS) at diagnosis were 0 ± 0.8 (0-3) and 20 ± 9.4 (7-45), respectively. Twenty-three patients were treated with cyclophosphamide (CYC) plus corticosteroids and the outcomes were similar between oral (17/23) and intravenous (6/23) CYC regimen. The mortality rate was 8% during a mean follow-up duration of 3.8 years and was associated with the initial FFS (p = 0.01), BVAS (p = 0.03), vasculitis damage index (p = 0.04), and presence of major organ involvements (P < 0.05) including GI and kidney.

Conclusions Granulomatosis with polyangiitis is an uncommon, multi-organ disorder that relapses frequently (45%). Refractory cases (17%) need prolonged immunosuppressant. Although the outcome has been significantly improved, it still causes permanent organ damages and chronic disability.

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Disclosure of Interest None Declared