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

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Objective: Adult-onset IgA vasculitis (Henoch-Schönlein) (IgAV) is a rare systemic vasculitis characterized 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 (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 IgAV. Methods: In this multicenter observational study, we included patients with adult-onset IgAV 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 IgAV.


  • Federica Maritati
  • Roberta Fenoglio
  • Evangeline Pillebout
  • Giacomo Emmi
  • Maria L. Urban
  • Rossana Rocco
  • Maria Nicastro
  • Monia Incerti
  • Matteo Goldoni
  • Giorgio Trivioli
  • Elena Silvestri
  • Aladdin J. Mohammad
  • David Jayne
  • Per Eriksson
  • Mårten Segelmark
  • Pavel Novikov
  • Helen Harris
  • Dario Roccatello
  • Augusto Vaglio
External organisations
  • Parma University Hospital
  • Saint-Louis Hospital, Paris
  • University of Florence
  • University of Parma
  • University of Cambridge
  • Linköping University
  • University of Turin
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity
Original languageEnglish
Pages (from-to)109-114
Number of pages6
JournalArthritis and Rheumatology
Issue number1
Publication statusPublished - 2018
Publication categoryResearch