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Published on: May 6, 2018
Rituximab in refractory nephrotic syndrome
Agnieszka Prytuła1, Kazumoto Iijima, Koichi Kamei
1Department of Paediatric Nephrology, Great Ormond Street Hospital-NHS Trust, London, UK.
Pediatric Nephrology (Berlin, Germany)
|December 25, 2009
Summary
Rituximab shows promise for refractory nephrotic syndrome (NS), particularly in steroid-dependent cases. Further controlled trials are needed to confirm its efficacy in idiopathic NS.
Area of Science:
- Pediatric Nephrology
- Immunology
- Pharmacology
Background:
- Refractory nephrotic syndrome (NS) poses significant treatment challenges in children.
- Existing therapies for steroid-dependent, steroid-resistant, and post-transplant recurrent NS have limitations.
Purpose of the Study:
- To evaluate the efficacy and safety of rituximab in pediatric patients with refractory nephrotic syndrome (NS).
- To assess rituximab's effectiveness across different subtypes of refractory NS: steroid-dependent/frequently relapsing, steroid-resistant, and post-transplant recurrence.
Main Methods:
- Retrospective data collection via questionnaires distributed to members of the International Paediatric Nephrology Association.
- Analysis of rituximab treatment outcomes in 105 pediatric patients across three distinct groups.
- Categorization of patients based on NS classification: steroid-dependent/frequently relapsing, steroid-resistant, and post-transplant recurrence.
Main Results:
- A good initial response to rituximab was observed in 82% of patients with steroid-dependent/frequently relapsing NS (Group 1).
- Responses in steroid-resistant NS (Group 2) and post-transplant recurrence (Group 3) were 44% and 60%, respectively.
- Adverse events occurred in 27% of patients, predominantly acute reactions.
Conclusions:
- Rituximab demonstrates a notable initial efficacy in pediatric refractory nephrotic syndrome, especially in steroid-dependent cases.
- The efficacy in post-transplant recurrence may be influenced by concurrent treatments.
- Controlled prospective trials are essential to definitively establish rituximab's therapeutic value in idiopathic NS.
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