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

Pediatric Nephrology volume 23, pages1269–1279(2008)Cite this article

Abstract

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 One or more immunosuppressive (IS) treatments could be withdrawn in 19 patients (85%), with no relapse of proteinuria and without increasing other IS drugs.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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Acknowledgements

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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Affiliations

  1. Department of Pediatrics, Centre de référence des maladies rénales rares du Sud-Ouest, Hôpital de la mère et de l’enfant, 8 Ave Dominique Larrey, 87000, Limoges, France

    Vincent Guigonis & Aymeric Dallocchio

  2. Pediatric Nephrology, Hôpital Robert Debré, AP-HP, Paris, France

    Véronique Baudouin

  3. Pediatric Nephrology, Hôpital Jeanne de Flandres, Lille, France

    Maud Dehennault

  4. Centre de référence des maladies rénales rares du Sud-Ouest, Pediatric Nephrology, Hôpital Purpan, Toulouse, France

    Caroline Hachon-Le Camus & François Bouissou

  5. Pediatric Nephrology, Hôpital l’Archet II, Nice, France

    Mickael Afanetti

  6. Pediatric Nephrology, Emma Children’s Hospital, Amsterdam, The Netherlands

    Jaap Groothoff

  7. Pediatric Nephrology, Centre de référence des maladies rénales rares du Sud-Ouest, Hôpital Pellegrin, Bordeaux, France

    Brigitte Llanas

  8. Pediatric Nephrology, Hôpital Necker Enfants Malades, AP-HP, Paris, France

    Patrick Niaudet

  9. Pediatric Nephrology, Hôpital Clocheville, Tours, France

    Hubert Nivet

  10. Pediatric Nephrology, Hôpital F Guyon, La Réunion, France

    Natacha Raynaud

  11. Pediatric Nephrology, Hôpital de Pontchaillou, Rennes, France

    Sophie Taque

  12. Department of Nephrology, INSERM, UMR S 702, UPMC Univ Paris 06, Hôpital Tenon, AP-HP, F-75020, Paris, France

    Pierre Ronco

  13. Centre de référence des maladies révales rares du Sud-Quest, Toulouse, France

    François Bouissou

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  1. Vincent Guigonis
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  2. Aymeric Dallocchio
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  12. Sophie Taque
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  13. Pierre Ronco
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  14. François Bouissou
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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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Keywords

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