Rituximab efficiency in children with steroid-dependent nephrotic syndrome

Pediatr Nephrol. 2010 Jun;25(6):1109-15. doi: 10.1007/s00467-010-1465-6. Epub 2010 Mar 18.

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.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Antibodies, Monoclonal / therapeutic use*
  • Antibodies, Monoclonal, Murine-Derived
  • B-Lymphocytes / drug effects*
  • Child
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunologic Factors / therapeutic use*
  • Immunosuppressive Agents / therapeutic use
  • Lymphocyte Count
  • Male
  • Nephrotic Syndrome / drug therapy*
  • Rituximab
  • Treatment Outcome

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunologic Factors
  • Immunosuppressive Agents
  • Rituximab