OBJECTIVE: Adult-onset IgA vasculitis (IgAV, formerly Henoch-Schönlein purpura) is a rare systemic vasculitis characterised by IgA1-dominant deposits. The treatment of adult-onset IgAV is controversial and is based on the combination of glucocorticoids and immunosuppressive agents, but many patients have refractory or relapsing disease despite treatment. Rituximab is a B cell-depleting antibody of proven efficacy in anti-neutrophil cytoplasmic antibody-associated vasculitis. We tested the efficacy and safety of rituximab in a multicentre cohort of patients with adult-onset IgAV. METHODS: In this multicentre, observational study we included patients with adult-onset IgAV who had received rituximab for either refractory/relapsing disease or because they had contraindications to conventional glucocorticoid/immunosuppressive therapy. We analysed the rates of remission (defined on the basis of the Birmingham Vasculitis Activity Score, BVAS) and relapse, and the variations over time in estimated glomerular filtration rate (eGFR), proteinuria, C-reactive protein (CRP) levels, BVAS and prednisone dose. RESULTS: Twenty-two patients were included; their median follow-up was 24 months (interquartile range, 18-48). Sixteen patients received rituximab as add-on therapy and six as monotherapy. Twenty patients (90.9%) achieved remission and seven (35%) subsequently relapsed. There was a significant reduction in 24h-proteinuria (p<0.0001), C-reactive protein (p=0.0005), BVAS (p<0.0001), and prednisone dose (p<0.0001) from rituximab initiation through last follow-up; eGFR remained stable. Rituximab was generally well tolerated. One patient died after 60 months of follow-up. CONCLUSIONS: Our data suggest that rituximab is an effective and safe therapeutic option for adult-onset IgAV. This article is protected by copyright. All rights reserved.
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