Costa Cláudia, Antunes Amélia, Oliveira João, Pereira Marta, Godinho Iolanda, Fernandes Paulo, Jorge Sofia, Lopes José António, Gameiro Joana
Serviço de Nefrologia e Transplantação Renal, Unidade Local de Saúde Santa Maria, Lisboa, Portugal.
Clínica Universitária de Nefrologia, Faculdade de Medicina da Universidade de Lisboa, Lisboa, Portugal.
Glomerular Dis. 2024 Jul 1;4(1):129-136. doi: 10.1159/000539922. eCollection 2024 Jan-Dec.
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.
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.
Fourteen patients (mean age at start of RTX 29.1 ± 21.9 years) with MCD ( = 7) or FSGS ( = 7) were treated with 2 doses of 1,000 mg 2 weeks apart ( = 13) or four doses of 375 mg/m ( = 1) of RTX. At last follow-up (mean 47.3 ± 101.7 months), 10 patients were in complete remission and two remained in partial remission. A reduction in the number of relapses, number of patients under corticosteroids and immunosuppressants, and dose of prednisolone was observed when compared to baseline (14 [100%] vs. 5 [35.7%]; 8/14 [57.1%] vs. 4/12 [33.3%]; 13/14 [92.9%] vs. 7/12 [58.3%]; 20 mg/day vs. 5.25 mg/day, respectively). Maintenance RTX therapy was used in 6 patients, with sustained complete remission. Infusion reactions were observed in 4 patients (one required treatment withdrawal).
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.
利妥昔单抗（RTX）已被报道为原发性微小病变病（MCD）和与类固醇依赖及频繁复发相关的局灶节段性肾小球硬化（FSGS）的有效治疗选择。然而，最佳的RTX治疗方案以及进一步剂量的RTX疗效仍不明确。本研究旨在评估诱导和维持RTX治疗在成人原发性足细胞病中的应用。
我们对接受诱导RTX治疗的类固醇依赖型足细胞病成年患者进行了一项回顾性病例系列研究。维持治疗由医生自行决定。分析缓解率和复发率、同时使用的皮质类固醇和免疫抑制剂、B细胞耗竭情况及不良事件。
14例患者（RTX开始时的平均年龄为29.1±21.9岁），其中7例为MCD，7例为FSGS，接受了2剂间隔2周的1000mg（13例）或4剂375mg/m²（1例）的RTX治疗。在最后一次随访时（平均47.3±101.7个月），10例患者完全缓解，2例仍部分缓解。与基线相比，复发次数、接受皮质类固醇和免疫抑制剂治疗的患者数量以及泼尼松龙剂量均有所减少（分别为14例[100%]对5例[35.7%]；8/14例[57.1%]对4/12例[33.3%]；13/14例[92.9%]对7/12例[58.3%]；20mg/天对5.25mg/天）。6例患者接受了维持RTX治疗，维持完全缓解。4例患者出现输液反应（1例需要停止治疗）。
我们的研究结果支持使用RTX实现足细胞病的无类固醇缓解，并表明维持RTX耐受性良好且与延长缓解期相关。需要进一步研究以证实其疗效和安全性，并确定类固醇依赖型足细胞病的最佳诱导和维持RTX治疗方案。
