Zhi Yuanzhao, Cao Lu, Gu Rui, Wang Qin, Shi Peipei, Zhu Lin, Cheung Wai W, Zhou Ping, Zhang Jianjiang
Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, Henan, China.
Department of Pediatric Nephrology and Rheumatology, Sichuan Provincial Maternity and Child Health Care Hospital, Chengdu, 610045, Sichuan, China.
Pediatr Nephrol. 2025 May;40(5):1635-1644. doi: 10.1007/s00467-024-06622-z. Epub 2025 Jan 4.
The effectiveness of rituximab (RTX) for steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) in children is well documented. However, there are insufficient data on relapse risk factors. Additionally, the retreat regimen for relapsed children requires further investigation.
We administered single dose RTX (375 mg/m, maximum 500 mg) to children with SDNS/FRNS between May 2020 and December 2022. An additional single dose of RTX was administered when B-cell depletion (CD19 + B cells < 1%) was incomplete or B-cell recovery (CD19 + B cells ≥ 1%) occurred. Primary and secondary outcomes were the first and second relapse, respectively.
Eighty-nine patients were included and the observation period was 12.2-43.2 months. Thirty-three patients (37.1%) relapsed after RTX treatment. Multivariate analysis showed that previous steroid-resistant nephrotic syndrome (SRNS) history and low NK-cell percentage at initial RTX treatment were independent risk factors for first relapse. In the relapse group, 26 patients (78.8%) continued RTX treatment upon B-cell recovery. During mean follow-up period of (15.4 ± 8.1) months, 15 patients (45.5%) experienced a second relapse. Compared with non-continued RTX treatment group, the continued RTX treatment group had a lower relapse rate (34.6% (9/26) versus 85.7% (6/7); P = 0.047) and fewer relapses (0.0 (0.0, 0.6) versus 1.8 (0.9, 2.7) times/year; P = 0.004). Multivariate analysis showed that continued RTX treatment was the protective factor for second relapse.
Previous SRNS history and low NK-cell percentage at initial RTX treatment may be associated with higher risk of relapse. Despite the possibility of relapse during RTX treatment, continued RTX treatment is effective in reducing relapse.
利妥昔单抗（RTX）治疗儿童激素依赖型/频繁复发型肾病综合征（SDNS/FRNS）的有效性已有充分记录。然而，关于复发危险因素的数据不足。此外，复发儿童的再治疗方案需要进一步研究。
2020年5月至2022年12月期间，我们对SDNS/FRNS患儿给予单剂量RTX（375mg/m²，最大500mg）。当B细胞清除不完全（CD19⁺B细胞＜1%）或B细胞恢复（CD19⁺B细胞≥1%）时，再给予单剂量RTX。主要和次要结局分别为首次和第二次复发。
纳入89例患者，观察期为12.2 - 43.2个月。33例患者（37.1%）在RTX治疗后复发。多因素分析显示，既往激素抵抗型肾病综合征（SRNS）病史和初始RTX治疗时NK细胞百分比低是首次复发的独立危险因素。在复发组中，26例患者（78.8%）在B细胞恢复时继续接受RTX治疗。在平均（15.4±8.1）个月的随访期内，15例患者（45.5%）出现第二次复发。与未继续RTX治疗组相比，继续RTX治疗组的复发率较低（34.6%（9/26）对85.7%（6/7）；P = 0.047），复发次数较少（0.0（0.0，0.6）次/年对1.8（0.9，2.7）次/年；P = 0.004）。多因素分析显示，继续RTX治疗是第二次复发的保护因素。
既往SRNS病史和初始RTX治疗时NK细胞百分比低可能与较高的复发风险相关。尽管RTX治疗期间可能复发，但继续RTX治疗可有效减少复发。
