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

  • Nephrology - Case Report
  • Published:
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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.

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Acknowledgments

We thank Dr Paul Thorner, pathologist at the Hospital for Sick Children in Toronto for the podocin and nephrin stains as well as Dr Diane Hebert, pediatric nephrologist at the Hospital for Sick Children in Toronto for sharing the planned protocol for treatment with rituximab for children with primary FSGS.

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Authors and Affiliations

  1. Department of Pediatrics, Children’s Hospital of Western Ontario, University of Western Ontario, 800 Commissioners Road East, London, ON, Canada, N6A 5W9

    Megha Suri, Ajay P. Sharma, Guido Filler & Joanne Grimmer

  2. McMaster University Undergraduate Medical Program, Hamilton, ON, Canada

    Kim Tran

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  1. Megha Suri
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Correspondence to Guido Filler.

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Suri, M., Tran, K., Sharma, A.P. et al. Remission of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab. Int Urol Nephrol 40, 807–810 (2008). https://doi.org/10.1007/s11255-008-9393-0

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  • DOI: https://doi.org/10.1007/s11255-008-9393-0

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