Pediatric Nephrology

, Volume 25, Issue 6, pp 1109–1115

Rituximab efficiency in children with steroid-dependent nephrotic syndrome

  • Anne-Laure Sellier-Leclerc
  • Marie-Alice Macher
  • Chantal Loirat
  • Valérie Guérin
  • Hervé Watier
  • Michel Peuchmaur
  • Véronique Baudouin
  • Georges Deschênes
Original Article

DOI: 10.1007/s00467-010-1465-6

Cite this article as:
Sellier-Leclerc, AL., Macher, MA., Loirat, C. et al. Pediatr Nephrol (2010) 25: 1109. doi:10.1007/s00467-010-1465-6

Abstract

Although most patients with idiopathic nephrotic syndrome (NS) respond to steroid treatment, development of steroid dependency may require a long-term multidrug therapy including steroid and calcineurin inhibitor. Rituximab was shown to allow a reduction of the doses of steroid and immunosuppressive drugs in those patients. In the present series, 22 patients with steroid-sensitive, but steroid-dependent nephrotic syndrome were treated with rituximab. Rituximab reduced B cell count down to an undetectable level in all patients. A second treatment was necessary in 18 patients in order to maintain B cell depletion for up to 18 months. B cell depletion lasted 4.9 to 26 months (mean 17.2 months). At last follow-up, 9 patients were in remission without oral steroid or calcineurin inhibitor, although B cell count had recovered for 2.9 to 17 months (mean 9.5 months). A remission under ongoing B cell depletion was observed in 10 other patients in the absence of oral steroid or calcineurin inhibitor. Rituximab failed in 2 patients and 1 refused any additional treatment, despite B cell recovery and relapse. Toxicity of rituximab was limited to reversible cytokine shock in 2 patients and reversible neutropenia in 1 patient. No severe infection was observed.

Keywords

Rituximab Idiopathic nephrotic syndrome CD19 B cell depletion Steroid dependency Immunosuppressive treatment 

Copyright information

© IPNA 2010

Authors and Affiliations

  • Anne-Laure Sellier-Leclerc
    • 1
  • Marie-Alice Macher
    • 1
  • Chantal Loirat
    • 1
  • Valérie Guérin
    • 2
  • Hervé Watier
    • 3
  • Michel Peuchmaur
    • 1
  • Véronique Baudouin
    • 1
  • Georges Deschênes
    • 1
  1. 1.Pediatric Nephrology Department, Hôpital Robert Debré, APHPUniversité Paris VIIParisFrance
  2. 2.Laboratory of Immunology, Hôpital Robert Debré, APHPUniversité Paris VIIParisFrance
  3. 3.Laboratory of Immunology, Pilot Centre for the Monitoring of Therapeutic AntibodiesCHRU ToursToursFrance

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