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논문분류	춘계학술대회 초록집
제목	Rituximab therapy in childhood steroid-resistant nephrotic syndrome: an international, multi-centre study
저자	Eugene Yu-hin Chan
출판정보	2024; 2024(1):
키워드
초록	Objectives: To evaluate the efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS). Methods: We conducted a retrospective cohort study at 28 paediatric nephrology centres from 19 countries in Asia, Europe, North America and Oceania. Children with SRNS treated with rituximab were analysed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [≥6 months (CNI-resistant) and <6 months]. Primary outcome was complete/partial remission (CR/PR) as defined by IPNA/KDIGO guidelines. Secondary outcomes included kidney survival and adverse events. Results: Two-hundred-forty-six children (age, 6.9±4.2 years; 136 boys; 57% focal segmental glomerulosclerosis, FSGS) were followed for 32.4 (IQR, 18.6-64.9) months after rituximab. All patients were in non-remission before rituximab. 146 and 100 children received CNIs for ≥ and <6 months before rituximab, respectively. Patients with CNI-resistant SRNS, compared to those treated with CNIs for <6 months, had lower rates of NS remission (CR/PR) at 6- (36% vs 52%) and 12-months (35% vs 55%) post-rituximab (ps<0.01). Among CNI-resistant patients, multivariable analysis demonstrated that subnephrotic-range proteinuria at rituximab (ORadj 5.6, 95%CI 1.1-37.1, p=0.049) and late SR (ORadj 2.6, 95%CI 1.1-6.2, p=0.03) were predictive of CR/PR at 12-months. Non-response to rituximab (HRadj 7.0, 95%CI 2.2-21.8, p<0.001), pre-existing CKD (HRadj 2.4, 95%CI 1.0-5.6, p=0.04), and FSGS (HRadj 4.3, 95%CI 1.4-13.2, p=0.01) were predictors for kidney failure and/or death. Adverse events occurred in 30.5% and most were mild. Conclusions: Rituximab enhances remission in a subset of children with SRNS. Late SR and subnephrotic-range proteinuria predict favourable response to rituximab in children with CNI-resistant SRNS.
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