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Cyclophosphamide and rituximab in frequently relapsing/steroid-dependent nephrotic syndrome

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Abstract

Background

Steroid-sensitive nephrotic syndrome is the most common form of nephrotic syndrome in childhood, defined by the response to treatment with glucocorticoids with consequent remission. While most children eventually experience spontaneous resolution of the disease, some have a difficult course with frequent relapses or steroid dependence nephrotic syndrome (FRSDNS). The consequent steroid toxicity often prompts administration of other immunosuppressive drugs, traditionally cyclophosphamide. Recently, rituximab has been reported as effective in this disorder, but long-term experience is lacking.

Methods

Retrospective note review of all children with FRSDNS treated with a first course of cyclophosphamide and/or rituximab in our center between December 2006 and April 2015. We reviewed time to first relapse after treatment, co-medications, and side effects.

Results

A total of 102 children were treated with cyclophosphamide (79) and/or rituximab (42). Of these, 34 received cyclophosphamide prior to rituximab. Median time to first relapse was 7 months after cyclophosphamide and 14 months after rituximab. Documented side effects of cyclophosphamide included neutropenia, hair loss, and hemorrhagic cystitis (1). Rituximab was associated with an allergic reaction at infusion in two patients.

Conclusions

Rituximab was used in children with the most difficult to treat FRSDNS, yet was associated with longer remission time and less side effects than cyclophosphamide. A randomized controlled trial is needed to directly compare these drugs.

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Acknowledgments

We are grateful to Dr. Rukshana Shroff for help with generating the figure. DB is a HEFCE Clinical reader.

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Authors and Affiliations

  1. Department of Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust London UK and UCL Institute of Child Health, London, WC1N 3JH, UK

    Hazel Webb, Graciana Jaureguiberry, Stephanie Dufek, Kjell Tullus & Detlef Bockenhauer

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  1. Hazel Webb
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  2. Graciana Jaureguiberry
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  3. Stephanie Dufek
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  4. Kjell Tullus
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Correspondence to Kjell Tullus.

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The authors declare no conflict of interest.

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Webb, H., Jaureguiberry, G., Dufek, S. et al. Cyclophosphamide and rituximab in frequently relapsing/steroid-dependent nephrotic syndrome. Pediatr Nephrol 31, 589–594 (2016). https://doi.org/10.1007/s00467-015-3245-9

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  • DOI: https://doi.org/10.1007/s00467-015-3245-9

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