Single Infusion of Rituximab for Persistent Steroid-Dependent Minimal-Change Nephrotic Syndrome After Long-Term Cyclosporine

Pediatr Nephrol. 2010 Mar;25(3):539-44. doi: 10.1007/s00467-009-1377-5. Epub 2010 Jan 5.

Abstract

Rituximab (RTX) has been successfully used as a rescue therapy in children with steroid-dependent nephrotic syndrome (SDNS). However, little is known regarding maintenance therapy after a successful response to RTX in such patients. The efficacy and safety of a single RTX infusion (375 mg/m(2)) were assessed in ten patients who had persistent SDNS associated with minimal-change disease (MCD) despite the long-term use of cyclosporine (CsA). The mean follow-up after RTX infusion was 17 months. Applying RTX resulted in a significant reduction in the mean prednisolone (PSL) dose from 0.39 +/-0.18 to 0.15 +/- 0.14 mg/kg per day. The mean 12-month relapse rates significantly decreased from 4.1 +/- 1.7 to 0.6 +/- 0.6. All but one patient who had continued CsA as maintenance therapy after a single RTX infusion were able to withdraw from PSL without any relapses during the study period, whereas the remaining five patients who discontinued CsA experienced relapses after CD19 cells re-emerged, leading to the reintroduction of CsA or an additional RTX infusion. Infusion reactions occurred in five of ten patients. These data indicate that a single RTX infusion may improve response to CsA in patients with persistent SDNS due to the phenomenon of secondary resistance to CsA.

MeSH terms

  • Adolescent
  • Anti-Inflammatory Agents / therapeutic use
  • Antibodies, Monoclonal / administration & dosage
  • Antibodies, Monoclonal / adverse effects
  • Antibodies, Monoclonal / therapeutic use*
  • Antibodies, Monoclonal, Murine-Derived
  • Antigens, CD19
  • Child
  • Child, Preschool
  • Cyclosporine / adverse effects
  • Cyclosporine / therapeutic use*
  • Drug Resistance
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / adverse effects
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Lymphocytes / immunology
  • Male
  • Nephrosis, Lipoid / drug therapy*
  • Prednisolone / therapeutic use
  • Recurrence
  • Rituximab
  • Steroids / therapeutic use
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Antigens, CD19
  • Immunosuppressive Agents
  • Steroids
  • Rituximab
  • Cyclosporine
  • Prednisolone