Characteristics and management of IgA vasculitis (Henoch-Schönlein purpura) in adults: Data from the 260 patients included in the IGAVAS survey

Objectives: Data on adult IgA vasculitis (IgAV) are scarce. This survey was designed to better define clinical spectrum and efficacy of treatments in this population. Methods: We analyzed data from 260 patients with IgAV included in a French multicenter retrospective survey. Results: Mean age at diagnosis was 50.1±18 years, and 63% were male. Baseline manifestations included purpura (100%), arthralgia (62%), glomerulonephritis (70%) or gastro-intestinal involvement (53%). Thirty percent showed at baseline renal failure. In univariate analysis, response was 80% (n=64/80) in patients treated with corticosteroids (CS) alone compared to 77% (n=23/30) in patients treated with cyclophosphamide (CYC) and 10/17 (59%) treated with colchicine (P=0.17). Multivariable analysis showed that patients treated with CS or CS plus CYC were more effective than colchicine in achieving response using different statistical analysis: logistic regression model [OR (95% CI) 3.68 (1.10-12.33), P=0.03], inverse probability weighting on Propensity Score (PS) [OR 3.75 (1.28-10.99), P=0.02]. Efficacy of CS plus CYC versus CS were discordant according to the method used. The use of multivariable logistic regression model [OR 0.88 (0.29-2.67), P=0.82] did not demonstrate difference, in contrast, inverse probability weighting on PS with [OR 1.79 (1.00-3.20), P=0.049] showed that CS plus CYC were more effective. Conclusion: This series constitutes the largest series reported so far in the literature of adults IgAV. It provides data on clinical and histological presentation and therapeutic efficacy, suggesting that CS alone appears to be a reasonable first-line therapy in patients with IgAV, while the benefit of adding CYC to CS remains uncertain. This article is protected by copyright. All rights reserved.