Wang Li, Hua Ran, Zhu Ying, Lu Yan, Gao Hui, Xia Xun, Zhang Qin, Lu Ling, Deng Fang
Department of Nephrology, Children's Hospital of Anhui Medical University, Anhui, China.
Iran J Kidney Dis. 2021 Mar;15(2):109-115.
Steroid-dependent (SD)/frequently relapsing (FR) nephrotic syndrome (NS) follows a relapsing and remitting course. It is also characterized by proteinuria and edema, which can significantly affect health-related quality of life (HRQOL) in children. This study evaluated the effectiveness and safety of a single dose of rituximab (RTX) as well as the impact of RTX on HRQOL in children with SDFRNS.
Sixteen children with SDFRNS were enrolled in the study. Each patient was administered a single intravenous dose of RTX (375 mg/m2). Effectiveness was defined as remission of proteinuria. The side effects of RTX were monitored. HRQOL was assessed using PedsQL™ 4.0 Generic Core Scales.
All the patients completed the study. Three SDNS patients and three FRNS patients discontinued treatment over 1 to 3.25 years of follow-up. Additionally, three SDNS patients and three FRNS patients experienced 1 to 2 relapses. The mean relapse-free period was 79.0 ± 77.6 days. The mean dosages of prednisolone and other immunosuppressants required were significantly lower (P < .05, < .001) six months after treatment with RTX compared with six months before treatment. Relapse rate was significantly reduced (P < .001) after treatment with RTX. Skin rash, hypotension, and fever were observed in one child. Total health score and physical, emotional, and school functioning were significantly higher six months after treatment with RTX (P < .001).
A single dose of RTX is effective and safe for children with SDFRNS and can improve HRQOL, especially physical, emotional, and school functioning.
激素依赖型（SD）/频繁复发型（FR）肾病综合征（NS）呈复发和缓解病程。其特征还包括蛋白尿和水肿，这会显著影响儿童的健康相关生活质量（HRQOL）。本研究评估了单剂量利妥昔单抗（RTX）治疗SD/FR NS患儿的有效性和安全性以及RTX对HRQOL的影响。
16例SD/FR NS患儿纳入本研究。每位患者静脉注射单剂量RTX（375 mg/m²）。有效性定义为蛋白尿缓解。监测RTX的副作用。使用儿童生活质量量表（PedsQL™）4.0通用核心量表评估HRQOL。
所有患者均完成研究。在1至3.25年的随访期间，3例SD NS患者和3例FR NS患者停止治疗。此外，3例SD NS患者和3例FR NS患者经历了1至2次复发。无复发的平均时间为79.0±77.6天。与治疗前6个月相比，RTX治疗后6个月泼尼松龙和其他免疫抑制剂的平均用量显著降低（P<.05，<.001）。RTX治疗后复发率显著降低（P<.001）。1例患儿出现皮疹、低血压和发热。RTX治疗后6个月，总体健康评分以及身体、情感和学校功能显著更高（P<.001）。
单剂量RTX治疗SD/FR NS患儿有效且安全，并可改善HRQOL，尤其是身体、情感和学校功能。
