Efficacy and Safety of Rituximab in Children With Steroid- and Cyclosporine-resistant and Steroid- and Cyclosporine-dependent Nephrotic Syndrome

Iran J Kidney Dis. 2018 Jan;12(1):27-32.

Abstract

Introduction: There is evidence of the effectiveness of rituximab in treatment of nephrotic syndrome in children. The present study aimed to assess safety and the therapeutic effectiveness of rituximab in steroid- and cyclosporine-resistant pediatric nephrotic syndrome.

Materials and methods: Forty-three children with steroid- and cyclosporine-resistant or steroid- and cyclosporine-dependent noncongenital nephrotic syndrome were included in the study to receive intravenous rituximab, 375 mg/m2/wk, for 4 weeks. The children were followed up for 2 years. Effectiveness was defined as remission of proteinuria in response to rituximab. Side effects of rituximab were monitored.

Results: Overall, 23 (57.1%) of the children had steroid- and cyclosporine-resistant nephrotic syndrome, of whom 8 (34.8%) revealed complete response and 3 (13%) revealed partial response. Seven children (16.7%) had late-resistant nephrotic syndrome, of whom 6 (85.7%) revealed complete response and none revealed partial response. Ten children (26.2%) had steroid- and cyclosporine-dependence all of whom revealed complete response to rituximab. Complete response rate was significantly higher in those with drug-dependent pattern than the other groups (P = .002). There was no association between response to rituximab and pathological basis of disease. Side effects were found in 4 patients as leukopenia in 2, alopecia in 1, and eosinophilia in 1.

Conclusions: Rituximab is effective for children with nephrotic syndrome with high efficacy and well tolerability, especially in those with steroid- and cyclosporine-dependent nephrotic syndrome.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Age Factors
  • Child
  • Child, Preschool
  • Cyclosporine / adverse effects
  • Cyclosporine / therapeutic use*
  • Female
  • Humans
  • Immunosuppressive Agents / adverse effects
  • Immunosuppressive Agents / therapeutic use*
  • Kidney / drug effects*
  • Kidney / pathology
  • Kidney / physiopathology
  • Male
  • Nephrotic Syndrome / congenital*
  • Nephrotic Syndrome / diagnosis
  • Nephrotic Syndrome / drug therapy
  • Nephrotic Syndrome / physiopathology
  • Proteinuria / diagnosis
  • Proteinuria / drug therapy
  • Proteinuria / physiopathology
  • Remission Induction
  • Rituximab / adverse effects
  • Rituximab / therapeutic use*
  • Steroids / adverse effects
  • Steroids / therapeutic use*
  • Time Factors
  • Treatment Outcome

Substances

  • Immunosuppressive Agents
  • Steroids
  • Rituximab
  • Cyclosporine

Supplementary concepts

  • Nephrotic syndrome, idiopathic, steroid-resistant