Brief Report

Pediatric Nephrology

, Volume 25, Issue 3, pp 539-544

First online:

Single infusion of rituximab for persistent steroid-dependent minimal-change nephrotic syndrome after long-term cyclosporine

  • Shuichiro FujinagaAffiliated withDivision of Nephrology, Saitama Children′s Medical Center Email author 
  • , Daishi HiranoAffiliated withDivision of Nephrology, Saitama Children′s Medical Center
  • , Naoto NishizakiAffiliated withDivision of Nephrology, Saitama Children′s Medical Center
  • , Koichi KameiAffiliated withDivision of Nephrology, National Center for Child Health and Development
  • , Shuichi ItoAffiliated withDivision of Nephrology, National Center for Child Health and Development
  • , Yoshiyuki OhtomoAffiliated withDepartment of Pediatrics, Juntendo Nerima Hospital
  • , Toshiaki ShimizuAffiliated withDepartment of Pediatrics, Juntendo University School of Medicine
  • , Kazunari KanekoAffiliated withDepartment of Pediatrics, Kansai Medical University

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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/m2) 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.

Keywords

Rituximab Steroid-dependent nephrotic syndrome Minimal-change disease Cyclosporine