International Urology and Nephrology

, Volume 40, Issue 3, pp 807–810

Remission of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab

  • Megha Suri
  • Kim Tran
  • Ajay P. Sharma
  • Guido Filler
  • Joanne Grimmer
Nephrology - Case Report

DOI: 10.1007/s11255-008-9393-0

Cite this article as:
Suri, M., Tran, K., Sharma, A.P. et al. Int Urol Nephrol (2008) 40: 807. doi:10.1007/s11255-008-9393-0

Abstract

Background

Therapeutic options are limited in cases of focal and segmental glomerulosclerosis (FSGS) that fail to respond to steroids and calcineurin inhibitors. We describe a case in which steroid-resistant nephrotic syndrome (SRNS) secondary to FSGS did not respond to conventional treatment, but was successfully treated with rituximab. Unlike previous reports in which rituximab was used in conjunction with a calcineurin inhibitor, we present the first case where rituximab was used as the sole therapeutic agent.

Case report

An 11-month-old boy presented with severe manifestations of primary nephrotic syndrome with a subsequent non-responsive steroid course. A renal biopsy confirmed FSGS, with normal staining for podocin and nephrin. Genetic studies for podocin were normal. The child developed ciclosporin-induced hemolytic uremic syndrome (HUS), and the response to plasma exchange, following ciclosporin withdrawal, was only temporary. A trial of a combination of mycophenolate and dexamethasone did not have any effect on proteinuria or fluid status. Four weekly rituximab infusions at 375 mg/m2/dose induced a complete remission without any adverse effects.

Conclusions

This case suggests that rituximab may be used as the sole therapeutic agent in the treatment of SRNS secondary to FSGS, especially in cases where calcineurin inhibitors are contraindicated.

Keywords

FSGS Steroid-resistant nephrotic syndrome Ciclosporin-induced atypical hemolytic uremic syndrome Plasma exchange Rituximab Mycophenolate mofetil 

Copyright information

© Springer Science+Business Media, B.V. 2008

Authors and Affiliations

  • Megha Suri
    • 1
  • Kim Tran
    • 2
  • Ajay P. Sharma
    • 1
  • Guido Filler
    • 1
  • Joanne Grimmer
    • 1
  1. 1.Department of Pediatrics, Children’s Hospital of Western OntarioUniversity of Western OntarioLondonCanada
  2. 2.McMaster University Undergraduate Medical ProgramHamiltonCanada