Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases


Several case reports suggest that rituximab (RTX) could be effective in steroid-dependent nephrotic syndrome, but RTX efficacy has not yet been studied in a series of patients. Safety and efficacy of RTX were assessed in a multicenter series of 22 patients aged 6.3–22 years with severe steroid-dependent nephrotic syndrome or steroid-resistant but cyclosporin-sensitive idiopathic nephrotic syndrome. Patients were treated with two to four infusions of RTX. Seven patients were nephrotic at the time of RTX treatment. Peripheral B cells were depleted in all subjects. Remission was induced in three of the seven proteinuric patients. One or more immunosuppressive (IS) treatments could be withdrawn in 19 patients (85%), with no relapse of proteinuria and without increasing other IS drugs. RTX was effective in all patients when administered during a proteinuria-free period in association with other IS agents. When relapses occurred, they were always associated with an increase in CD19 cell count. Adverse effects were observed in 45% of cases, but most of them were mild and transient. This study suggests that RTX could be an effective treatment for severe steroid-dependent nephrotic syndrome.

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We are grateful to Pr. Thibault (Tours, France), Pr. Deschênes (Paris, France) and Dr. François (Paris, France) for helpful discussions and to C. Bahans for proofreading the manuscript.

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Correspondence to Vincent Guigonis.

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V. Guigonis and A. Dallocchio contributed equally to this article

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Guigonis, V., Dallocchio, A., Baudouin, V. et al. Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases. Pediatr Nephrol 23, 1269–1279 (2008). https://doi.org/10.1007/s00467-008-0814-1

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  • Idiopathic nephrotic syndrome
  • Rituximab
  • CD19
  • B-cell depletion