Abstract：
BACKGROUND: The efficacy of rituximab in steroid-resistant nephrotic syndrome (SRNS) is controversial. We previously reported that rituximab in combination with methylprednisolone pulse therapy (MPT) and immunosuppressants was associated with favorable outcomes. We determined risk factors for poor response following rituximab treatment, which remains unknown.
METHODS: This retrospective study included 45 patients with childhood-onset SRNS treated with rituximab across four pediatric kidney facilities. Treatment effects were categorized as complete remission (CR), partial remission (PR), and no remission (NR) at one year after rituximab treatment. The primary outcome was the rate of CR, PR, and NR. Risk factors for non-CR were calculated with multivariate logistic regression. Adverse events and the relationship between disease status at one year and long-term prognosis were also evaluated.
RESULTS: The rates of CR, PR, and NR at one year were 69%, 24%, and 7%, respectively. The median time from rituximab administration to CR was 90 days. The median follow-up period after rituximab administration was 7.4 years. In multivariate analysis, significant risk factors for poor response were the pathologic finding of focal segmental glomerular sclerosis and a long interval between SRNS diagnosis and rituximab administration. The rates of CR were 90.3% and 21.4% in patients receiving rituximab within and after 6 months following SRNS diagnosis, respectively (p < 0.001). Five patients developed chronic kidney disease stage G5, including 2 of the 11 patients with PR and all 3 patients with NR, whereas none of the 31 patients with CR developed chronic kidney disease stage G5.
CONCLUSIONS: Early administration of rituximab in combination with MPT and immunosuppressants might achieve favorable outcomes in patients with SRNS.
METHODS: This retrospective study included 45 patients with childhood-onset SRNS treated with rituximab across four pediatric kidney facilities. Treatment effects were categorized as complete remission (CR), partial remission (PR), and no remission (NR) at one year after rituximab treatment. The primary outcome was the rate of CR, PR, and NR. Risk factors for non-CR were calculated with multivariate logistic regression. Adverse events and the relationship between disease status at one year and long-term prognosis were also evaluated.
RESULTS: The rates of CR, PR, and NR at one year were 69%, 24%, and 7%, respectively. The median time from rituximab administration to CR was 90 days. The median follow-up period after rituximab administration was 7.4 years. In multivariate analysis, significant risk factors for poor response were the pathologic finding of focal segmental glomerular sclerosis and a long interval between SRNS diagnosis and rituximab administration. The rates of CR were 90.3% and 21.4% in patients receiving rituximab within and after 6 months following SRNS diagnosis, respectively (p < 0.001). Five patients developed chronic kidney disease stage G5, including 2 of the 11 patients with PR and all 3 patients with NR, whereas none of the 31 patients with CR developed chronic kidney disease stage G5.
CONCLUSIONS: Early administration of rituximab in combination with MPT and immunosuppressants might achieve favorable outcomes in patients with SRNS.
摘要：
背景：利妥昔单抗治疗类固醇耐药型肾病综合征（SRNS）的疗效存在争议。我们先前报道，利妥昔单抗联合甲基强的松龙冲击疗法（MPT）和免疫抑制剂与良好的预后相关。我们确定了利妥昔单抗治疗后反应不良的危险因素，仍然未知。
方法：这项回顾性研究纳入了45例儿童期发病的SRNS患者，这些患者在4个儿科肾脏机构中接受了利妥昔单抗治疗。治疗效果被归类为完全缓解（CR），部分缓解（PR），利妥昔单抗治疗后一年无缓解（NR）。主要结果是CR率，PR,和NR。采用多因素logistic回归计算非CR的危险因素。还评估了不良事件以及一年时疾病状态与长期预后之间的关系。
结果：CR率，PR,一年的NR为69%，24%,7%,分别。从利妥昔单抗给药到CR的中位时间为90天。利妥昔单抗给药后的中位随访期为7.4年。在多变量分析中，反应不良的重要危险因素是局灶性节段性肾小球硬化的病理发现以及SRNS诊断和利妥昔单抗给药之间的间隔较长.在SRNS诊断后6个月内和之后接受利妥昔单抗的患者的CR率分别为90.3%和21.4%。分别(p<0.001)。5例患者发展为慢性肾脏病G5期，包括11例患者中的2例PR和3例NR，而31例CR患者均未出现慢性肾脏病G5期。
结论：在SRNS患者中，早期给予利妥昔单抗联合MPT和免疫抑制剂可能取得良好的预后。
方法：这项回顾性研究纳入了45例儿童期发病的SRNS患者，这些患者在4个儿科肾脏机构中接受了利妥昔单抗治疗。治疗效果被归类为完全缓解（CR），部分缓解（PR），利妥昔单抗治疗后一年无缓解（NR）。主要结果是CR率，PR,和NR。采用多因素logistic回归计算非CR的危险因素。还评估了不良事件以及一年时疾病状态与长期预后之间的关系。
结果：CR率，PR,一年的NR为69%，24%,7%,分别。从利妥昔单抗给药到CR的中位时间为90天。利妥昔单抗给药后的中位随访期为7.4年。在多变量分析中，反应不良的重要危险因素是局灶性节段性肾小球硬化的病理发现以及SRNS诊断和利妥昔单抗给药之间的间隔较长.在SRNS诊断后6个月内和之后接受利妥昔单抗的患者的CR率分别为90.3%和21.4%。分别(p<0.001)。5例患者发展为慢性肾脏病G5期，包括11例患者中的2例PR和3例NR，而31例CR患者均未出现慢性肾脏病G5期。
结论：在SRNS患者中，早期给予利妥昔单抗联合MPT和免疫抑制剂可能取得良好的预后。
