Pediatric Nephrology

, Volume 28, Issue 2, pp 257–264

Survey of rituximab treatment for childhood-onset refractory nephrotic syndrome

Authors

    • Division of Pediatric Nephrology and RheumatologyNational Center for Child Health and Development
    • Division of Nephrology and RheumatologyNational Center for Child Health and Development
  • Koichi Kamei
    • Division of Pediatric Nephrology and RheumatologyNational Center for Child Health and Development
  • Masao Ogura
    • Division of Pediatric Nephrology and RheumatologyNational Center for Child Health and Development
  • Tomohiro Udagawa
    • Division of Pediatric Nephrology and RheumatologyNational Center for Child Health and Development
  • Shuichiro Fujinaga
    • Division of NephrologySaitama Children’s Medical Center
  • Mari Saito
    • Clinical Research CenterNational Center for Child Health and Development
  • Mayumi Sako
    • Clinical Research CenterNational Center for Child Health and Development
  • Kazumoto Iijima
    • Division of Child Health & Development, Department of PediatricsKobe University Graduate School of Medicine
Original Article

DOI: 10.1007/s00467-012-2319-1

Cite this article as:
Ito, S., Kamei, K., Ogura, M. et al. Pediatr Nephrol (2013) 28: 257. doi:10.1007/s00467-012-2319-1

Abstract

Background

Rituximab (RTX) is a promising option for treating childhood-onset steroid-dependent (SDNS), frequently relapsing (FRNS), and steroid-resistant (SRNS) nephrotic syndrome.

Methods

We retrospectively surveyed RTX treatment for these conditions to evaluate its indications, efficacy and adverse events. Questionnaires were sent to 141 hospitals in Japan.

Results

Seventy-four patients (52 SDNS; 3 FRNS; 19 SRNS) were treated with RTX because of resistance to various immunosuppressive agents. Most patients received a single administration of RTX (85%). Forty-one of 53 SDNS/FRNS (77%) and 5 of 17 SRNS (29%) patients successfully discontinued prednisolone (16 SDNS/FRNS and 6 SRNS achieved their first discontinuation since onset), and 17 out of 53 SDNS/FRNS patients (31%) discontinued cyclosporine. However, 28 of the 53 patients (51%) relapsed. Although immunosuppressive agents did not extend B cell depletion, relapses were significantly less if immunosuppressive agents were continued after RTX (P = 0.006; hazard ratio = 0.2). Among the SRNS patients, complete (n = 6) and partial remission (n = 6) were achieved. No life-threatening adverse events were experienced.

Conclusions

Although this was a multi-center survey where treatment of nephrotic syndrome varied between centers, the steroid-sparing effect of RTX in SDNS/FRNS was excellent. If single administration of RTX is chosen, continuation of immunosuppressive agents is recommended for prevention of relapse.

Keywords

RituximabNephrotic syndromeChildrenSteroidSteroid-dependent nephrotic syndromeSteroid-resistant nephrotic syndromeImmunosuppressive agents

Copyright information

© IPNA 2012