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Rituximab therapy for primary podocytopathies
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Introdução: O rituximab (RTX) tem sido descrito como uma alternativa terapêutica eficaz nas formas primárias de doença de lesões mínimas (DLM) e glomerulosclerose focal e segmentar (GEFS), associadas a corticodependência e recidivas frequentes. Contudo, pouco se sabe sobre o regime terapêutico adequado e os efeitos de doses adicionais de RTX. Este estudo teve como objetivo avaliar o uso da terapêutica de indução e manutenção com RTX nas podocitopatias primárias.
Métodos: Realizámos um estudo retrospetivo em adultos com podocitopatias corticodependentes, que receberam terapêutica de indução com RTX. A terapêutica de manutenção foi realizada conforme decisão do médico assistente. Foram analisadas as taxas de remissão e recidiva, o uso concomitante de corticosteroides e outros imunossupressores, a depleção de células B e os efeitos adversos.
Resultados: Foram incluídos 14 doentes com DLM (n=7) e GEFS (n=7), que receberam 2 doses de 1g com 2 semanas de intervalo (n=13) ou 4 doses de 375 mg/m2 de superfície corporal (n=1) de RTX. Aos 6 e 12 meses, 71.4% e 58.3% encontravam-se em remissão completa, e 14.3% e 33.3% em remissão parcial, respetivamente. Com um follow-up mediano de 32.5 meses (IIQ 32–62), observou-se uma redução no número de doentes com recidiva, sob corticosteroides e imunossupressores, e na dose de prednisolona, relativamente ao basal [14 (100%) vs 5 (35.7%); 8/14 (57.1%) vs 4/13 (30.8%); 13/14 (92.9%) vs 8/13 (61.5%); 20mg/dia vs 5.25mg/dia, respetivamente]. 6 doentes realizaram manutenção com RTX, com remissão completa sustentada. 4 doentes desenvolveram reações de infusão (1 necessitou de suspensão terapêutica).
Conclusão: Este estudo apoia o uso de RTX para uma remissão livre de corticóides em podocitopatias e sugere que a terapêutica de manutenção com RTX é bem tolerada e associa-se a uma remissão prolongada. São necessários estudos adicionais para esclarecer sobre a estratégia terapêutica ideal de indução e manutenção com RTX em podocitopatias corticodependentes.
Background: Rituximab (RTX) has been reported as an effective treatment alternative in primary forms of minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) associated with steroid dependence and frequent relapses. However, the optimal RTX regimen and the outcomes of further doses of RTX remain unclear. This study aimed to evaluate the use of induction and maintenance RTX therapy for adults with primary podocytopathies. Methods: We performed a retrospective case series on adult patients with steroid-dependent podocytopathies who received an induction RTX therapy. Maintenance therapy was performed at physician’s discretion. Remission and relapse rates, concomitant corticosteroids and immunosuppressants use, B-cell depletion and adverse events were analyzed. Results: Fourteen patients with MCD (n=7) or FSGS (n=7) were treated with 2 doses of 1g 2 weeks apart (n=13) or 4 doses of 375 mg/m2 body surface area (n=1) of RTX. At 6 and 12 months, 71.4% and 58.3% were in complete remission, and 14.3% and 33.3% in partial remission, respectively. A reduction in the number of relapses, number of patients under corticosteroids and immunosuppressants, and dose of prednisolone was observed at last follow-up (32.5 months, IQR 32–62), when compared to baseline [14 (100%) vs 5 (35.7%); 8/14 (57.1%) vs 4/13 (30.8%); 13/14 (92.9%) vs 8/13 (61.5%); 20 mg/day vs 5.25 mg/day, respectively]. Maintenance RTX therapy was used in six patients, with sustained complete remission. Infusion reactions were observed in four patients (one required treatment withdrawal). Conclusions: Our findings support the use of RTX for a steroid-free remission in podocytopathies and suggest that maintenance RTX is well-tolerated and associated with prolonged remission. Further studies are needed to confirm its efficacy and safety and establish the optimal induction and maintenance RTX regimen in steroid-dependent podocytopathies.
Background: Rituximab (RTX) has been reported as an effective treatment alternative in primary forms of minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) associated with steroid dependence and frequent relapses. However, the optimal RTX regimen and the outcomes of further doses of RTX remain unclear. This study aimed to evaluate the use of induction and maintenance RTX therapy for adults with primary podocytopathies. Methods: We performed a retrospective case series on adult patients with steroid-dependent podocytopathies who received an induction RTX therapy. Maintenance therapy was performed at physician’s discretion. Remission and relapse rates, concomitant corticosteroids and immunosuppressants use, B-cell depletion and adverse events were analyzed. Results: Fourteen patients with MCD (n=7) or FSGS (n=7) were treated with 2 doses of 1g 2 weeks apart (n=13) or 4 doses of 375 mg/m2 body surface area (n=1) of RTX. At 6 and 12 months, 71.4% and 58.3% were in complete remission, and 14.3% and 33.3% in partial remission, respectively. A reduction in the number of relapses, number of patients under corticosteroids and immunosuppressants, and dose of prednisolone was observed at last follow-up (32.5 months, IQR 32–62), when compared to baseline [14 (100%) vs 5 (35.7%); 8/14 (57.1%) vs 4/13 (30.8%); 13/14 (92.9%) vs 8/13 (61.5%); 20 mg/day vs 5.25 mg/day, respectively]. Maintenance RTX therapy was used in six patients, with sustained complete remission. Infusion reactions were observed in four patients (one required treatment withdrawal). Conclusions: Our findings support the use of RTX for a steroid-free remission in podocytopathies and suggest that maintenance RTX is well-tolerated and associated with prolonged remission. Further studies are needed to confirm its efficacy and safety and establish the optimal induction and maintenance RTX regimen in steroid-dependent podocytopathies.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2024
Palavras-chave
Podocitopatias Doença das lesões mínimas Glomeruloesclerose focal e segmentar Síndrome nefrótico corticodependente Rituximab Nefrologia