Pediatric Nephrology

, 23:1269

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

  • Vincent Guigonis
  • Aymeric Dallocchio
  • Véronique Baudouin
  • Maud Dehennault
  • Caroline Hachon-Le Camus
  • Mickael Afanetti
  • Jaap Groothoff
  • Brigitte Llanas
  • Patrick Niaudet
  • Hubert Nivet
  • Natacha Raynaud
  • Sophie Taque
  • Pierre Ronco
  • François Bouissou
Original Article

DOI: 10.1007/s00467-008-0814-1

Cite this article as:
Guigonis, V., Dallocchio, A., Baudouin, V. et al. Pediatr Nephrol (2008) 23: 1269. doi:10.1007/s00467-008-0814-1

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 One or more immunosuppressive (IS) treatments could be withdrawn in 19 patients (85%), with no relapse of proteinuria and without increasing other IS drugs.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

Keywords

Idiopathic nephrotic syndrome Rituximab CD19 B-cell depletion 

Copyright information

© IPNA 2008

Authors and Affiliations

  • Vincent Guigonis
    • 1
  • Aymeric Dallocchio
    • 1
  • Véronique Baudouin
    • 2
  • Maud Dehennault
    • 3
  • Caroline Hachon-Le Camus
    • 4
  • Mickael Afanetti
    • 5
  • Jaap Groothoff
    • 6
  • Brigitte Llanas
    • 7
  • Patrick Niaudet
    • 8
  • Hubert Nivet
    • 9
  • Natacha Raynaud
    • 10
  • Sophie Taque
    • 11
  • Pierre Ronco
    • 12
  • François Bouissou
    • 4
    • 13
  1. 1.Department of Pediatrics, Centre de référence des maladies rénales rares du Sud-OuestHôpital de la mère et de l’enfantLimogesFrance
  2. 2.Pediatric NephrologyHôpital Robert DebréParisFrance
  3. 3.Pediatric NephrologyHôpital Jeanne de FlandresLilleFrance
  4. 4.Centre de référence des maladies rénales rares du Sud-Ouest, Pediatric NephrologyHôpital PurpanToulouseFrance
  5. 5.Pediatric NephrologyHôpital l’Archet IINiceFrance
  6. 6.Pediatric NephrologyEmma Children’s HospitalAmsterdamThe Netherlands
  7. 7.Pediatric Nephrology, Centre de référence des maladies rénales rares du Sud-OuestHôpital PellegrinBordeauxFrance
  8. 8.Pediatric NephrologyHôpital Necker Enfants MaladesParisFrance
  9. 9.Pediatric NephrologyHôpital ClochevilleToursFrance
  10. 10.Pediatric NephrologyHôpital F GuyonLa RéunionFrance
  11. 11.Pediatric NephrologyHôpital de PontchaillouRennesFrance
  12. 12.Department of Nephrology, INSERM, UMR S 702UPMC Univ Paris 06, Hôpital Tenon, AP-HPParisFrance
  13. 13.Centre de référence des maladies révales rares du Sud-QuestToulouseFrance

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