#2789 Rituximab in treating steroid-sensitive nephrotic syndrome in children—experience from Uzbekistan

Abstract Background and Aims Steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS) significantly affect children's quality of life. While the majority of children with nephrotic syndrome respond to corticosteroids, half of these patients develop frequent relapses (FR) and/or steroid dependency (SD) and require multiple immunosuppressive agents, either in sequence or in combination, to prevent relapse. These children are at high risk of significant medication side effects such as short stature, Cushing syndrome, infection, and calcineurin inhibitor-associated nephrotoxicity. Rituximab (RTX), a chimeric anti-CD monoclonal antibody, has emerged as an effective therapy in children with FR-SD-NS to induce long-term disease remission and avoid steroid toxicities. Considering the challenges and adverse effects of previous treatments, rituximab has evolved into a therapy that can be used in different forms of nephrotic syndrome to induce long-term immunosuppression. The aim of this study is to find out the response of rituximab in idiopathic nephrotic syndrome and to look for response at 6, 9, 12, and 24 months after therapy. Method We conducted a prospective observational study in the Department of Paediatric Nephrology, National Children's Medical Centre, Uzbekistan, from May 2021 to December 2024, involving 86 children aged 3–18 with SSNS undergoing 3 years of continuous RTX therapy. The primary outcome was complete remission (CR), as defined by IPNA/KDIGO guidelines, at 6, 12, 18, and 24 months on RTX; secondary outcomes included adverse events. Kidney biopsies were performed in 65% of patients. RTX was administered every 6–12 months, depending on CD20 levels, IgG levels, and the presence of infections. The effectiveness of RTX was evaluated using eGFR (Schwarz formula) and proteinuria (ACR). The efficacy of RTX was assessed at three intervals: immediate (6 months post-therapy), short-term (12 months), and long-term (24 months). Results 86 children were selected for RTX treatment. Overall, 1 experienced a severe allergic reaction. 72% of children with SSNS achieved and maintained complete remission within 2 years. Remission rates in SSNS children ranged from 60% (RR 0.78; 95% CI: 16.4–61.4%) at the 6th month. At the end of 12 months, in the FRNS category, 90% of patients were in remission; in the SDNS category, 91.6% of patients were in remission. The total number of relapses in 1 year before giving rituximab and one year after giving rituximab was assessed, and it was seen that the number of relapses was significantly reduced after giving rituximab in each category with p-values of 0.000 and 0.000 in FRNS and SDNS. The median duration of RTX use was 26.1 months, with a median cumulative dose of 2950 mg/m². Adverse reactions and complications were presented by mild infusion-related reactions in 3 children (3.49%), severe allergic reactions in 1 child (1.16%), hypogammaglobulinemia in 4 children (4.65%), and infections in 2 children (2.32%). Conclusion RTX was tolerated well and proved highly effective as a steroid-sparing agent, offering potential in terms of stopping relapses and minimising steroid-related side effects. It also demonstrated efficacy in slowing progression in SRNS, indicating potential for use in ACR reduction and renal function restoration, but requires careful use given potential severe allergic reactions and infectious complications. Further studies should focus on long-term cost-effectiveness and deferred side effects.

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