Pediatric Nephrology

, Volume 23, Issue 3, pp 481–485

Rituximab for refractory focal segmental glomerulosclerosis

  • Makiko Nakayama
  • Koichi Kamei
  • Kandai Nozu
  • Kentaro Matsuoka
  • Atsuko Nakagawa
  • Mayumi Sako
  • Kazumoto Iijima
Brief Report

DOI: 10.1007/s00467-007-0640-x

Cite this article as:
Nakayama, M., Kamei, K., Nozu, K. et al. Pediatr Nephrol (2008) 23: 481. doi:10.1007/s00467-007-0640-x
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Abstract

We present the cases of two children with steroid-resistant nephrotic syndrome (SRNS) who were treated with rituximab (anti-CD20 monoclonal antibody). Both were resistant to conventional therapy, and renal biopsy showed focal segmental glomerulosclerosis (FSGS). Combination therapy with methylprednisolone pulse therapy and plasmapheresis was the only way to decrease proteinuria. However, the patients suffered severe reactions to steroid treatment. We therefore treated them with rituximab in a single dose of 375 mg/m2, which resulted in the rapid clearing of circulating CD19-positive B cells. One month after rituximab treatment, both achieved partial remission; one patient has maintained complete remission for 8 months, and the other relapsed 8 months after the first rituximab treatment with the recovery of peripheral B-cell counts and received a second rituximab treatment. She achieved complete remission 5 months after the second course and has maintained the remission for 2 months. We conclude that rituximab may be an effective treatment for refractory SRNS with FSGS.

Keywords

Steroid-resistant nephrotic syndromeFocal segmental glomerulosclerosisPlasmapheresisRituximabB cells

Copyright information

© IPNA 2007

Authors and Affiliations

  • Makiko Nakayama
    • 1
  • Koichi Kamei
    • 1
  • Kandai Nozu
    • 2
  • Kentaro Matsuoka
    • 3
  • Atsuko Nakagawa
    • 3
  • Mayumi Sako
    • 1
  • Kazumoto Iijima
    • 1
  1. 1.Department of Nephrology, National Children’s Medical CenterNational Center for Child Health and DevelopmentTokyoJapan
  2. 2.Department of PediatricsKobe University Graduate School of MedicineKobeJapan
  3. 3.Department of PathologyNational Center for Child Health and DevelopmentTokyoJapan