Katsuno Takayuki, Masuda Tomohiro, Saito Shoji, Kato Noritoshi, Ishimoto Takuji, Kato Sawako, Kosugi Tomoki, Tsuboi Naotake, Kitamura Hiroshi, Tsuzuki Toyonori, Ito Yasuhiko, Maruyama Shoichi
Department of Nephrology and Rheumatology, Aichi Medical University, 1-1 Karimata, Yazako, Nagakute, 480-1195, Japan.
Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Clin Exp Nephrol. 2019 Feb;23(2):207-214. doi: 10.1007/s10157-018-1630-y. Epub 2018 Aug 18.
Recent reports have described the efficacy of rituximab in treating steroid-dependent nephrotic syndrome (SDNS) in pediatric patients. However, few reports describe data regarding adult-onset SDNS. We investigated the efficacy of rituximab for the management of adult-onset SDNS.
We performed a retrospective cohort study investigating eight patients with adult-onset SDNS who were treated with rituximab. Clinical data were obtained at the initiation of rituximab therapy. The primary outcomes evaluated were successful suppression of relapses and CD19+ cells after rituximab treatment. The corticosteroid- and immunosuppressant-sparing effect and adverse events were additionally evaluated.
All eight patients were diagnosed with minimal change nephrotic syndrome and received immunosuppressants in addition to corticosteroid. Total number of relapses was 10.5 times as a median value. Rituximab administration was repeated in two patients, whereas six received single-dose rituximab. Three of eight (37.5%) patients showed relapse after rituximab therapy. A rituximab-induced depletion in CD19+ cells noted initially was followed by their reappearance in all patients. There were cases with no relapse after the reappearance of CD19+ cells. The median relapse time pre- and post-rituximab therapy showed a decrease from 1 time/year (interquartile range [IQR] 1-3 times/year) to 0 time/year (IQR 0-1 time/year). Rituximab treatment induced a significant reduction in the required doses of corticosteroid and cyclosporine (P < 0.01). No serious adverse events were observed.
Rituximab treatment was effective not only in childhood-onset but also in adult-onset SDNS. Further studies are needed to establish optimal treatment regimens.
近期报告描述了利妥昔单抗治疗小儿类固醇依赖型肾病综合征（SDNS）的疗效。然而，很少有报告描述成人发病型SDNS的数据。我们研究了利妥昔单抗治疗成人发病型SDNS的疗效。
我们进行了一项回顾性队列研究，调查了8例接受利妥昔单抗治疗的成人发病型SDNS患者。在开始利妥昔单抗治疗时获取临床数据。评估的主要结局是利妥昔单抗治疗后成功抑制复发和CD19+细胞。此外还评估了皮质类固醇和免疫抑制剂的减量效果及不良事件。
所有8例患者均被诊断为微小病变型肾病综合征，除皮质类固醇外还接受了免疫抑制剂治疗。复发总数中位数为10.5次。2例患者重复使用利妥昔单抗，而6例接受单剂量利妥昔单抗。8例患者中有3例（37.5%）在利妥昔单抗治疗后复发。最初观察到利妥昔单抗诱导CD19+细胞耗竭，随后所有患者的CD19+细胞重新出现。CD19+细胞重新出现后有未复发的病例。利妥昔单抗治疗前后复发时间中位数从每年1次（四分位间距[IQR]为每年1 - 3次）降至每年0次（IQR为每年0 - 1次）。利妥昔单抗治疗使皮质类固醇和环孢素的所需剂量显著减少（P < 0.01）。未观察到严重不良事件。
利妥昔单抗治疗不仅对儿童发病型SDNS有效，对成人发病型SDNS也有效。需要进一步研究以确定最佳治疗方案。
