Brief Report: Rituximab for the Treatment of Adult-Onset IgA Vasculitis (Henoch-Schönlein).

Objective Adult-onset IgA vasculitis (Henoch-Schönlein) (Ig AV) is a rare systemic vasculitis characterized by IgA1-dominant deposits. The treatment of adult-onset Ig AV is controversial and is based on the combination of glucocorticoids and immunosuppressive agents, but many patients have refractory or relapsing disease despite treatment. Rituximab ( RTX) is a B cell-depleting antibody of proven efficacy in antineutrophil cytoplasmic antibody-associated vasculitis. We undertook this study to test the efficacy and safety of RTX in a multicenter cohort of patients with adult-onset Ig AV. Methods In this multicenter observational study, we included patients with adult-onset Ig AV who had received RTX either for refractory/relapsing disease or because they had contraindications to conventional glucocorticoid/immunosuppressive therapy. We analyzed the rates of remission (defined on the basis of the Birmingham Vasculitis Activity Score [ BVAS]) and relapse as well as the variations over time in estimated glomerular filtration rate ( GFR), proteinuria, C-reactive protein ( CRP) level, BVAS, and prednisone dose. Results Twenty-two patients were included; their median duration of follow-up was 24 months (interquartile range 18-48 months). Sixteen patients received RTX as add-on therapy and 6 as monotherapy. Twenty patients (90.9%) achieved remission, and 7 of those 20 patients (35%) had subsequent relapse of disease. There were significant reductions in 24-hour proteinuria ( P < 0.0001), CRP level ( P = 0.0005), BVAS ( P < 0.0001), and prednisone dose ( P < 0.0001) from RTX initiation through the last follow-up visit; estimated GFR remained stable. RTX was generally well tolerated. One patient died after 60 months of follow-up. Conclusion Our data suggest that RTX is an effective and safe therapeutic option for adult-onset Ig AV. [ABSTRACT FROM AUTHOR]