Kamei Koichi, Ogura Masao, Sato Mai, Sako Mayumi, Iijima Kazumoto, Ito Shuichi
Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Division for Clinical Trials, Department of Development Strategy, Center for Social and Clinical Research, National Research Institute for Child Health and Development, National Center for Child Health and Development, Tokyo, Japan.
Pediatr Nephrol. 2016 Jan;31(1):89-95. doi: 10.1007/s00467-015-3197-0. Epub 2015 Sep 4.
Rituximab (RTX) is known to be effective for the treatment of refractory steroid-dependent nephrotic syndrome (SDNS). However, there are insufficient data on the risk factors for relapse and long-term outcome after RTX treatment.
We administered a single dose of RTX to patients with refractory SDNS from November 2007 to December 2013 and continued with immunosuppressants. The risk factors for early relapse and long-term outcome were analyzed.
Eighty-one patients were included and the observation period was 13-90 months. Seventy-six patients (94 %) discontinued steroids. Median duration of B-cell depletion was 160 days and 50 % relapse-free survival was 482 days. In multivariate analyses, only a history of steroid-resistant nephrotic syndrome (SRNS) was a statistically significant risk factor (hazard ratio, 2.44; p = 0.048). Fifty percent relapse-free survival in patients without a history of SRNS was 615 days, longer than that of patients with a history of SRNS (393 days) (p=0.005) [corrected]. Fifty-one patients (63 %) received additional RTX treatments for relapses. At last observation, patients using calcineurin inhibitors decreased from 89 % to 23 %, and 12 patients (15 %) discontinued immunosuppressants.
Rituximab treatment followed by immunosuppressants is an effective option for patients with SDNS, although a history of SRNS is a risk factor for early relapse.
已知利妥昔单抗(RTX)对治疗难治性激素依赖型肾病综合征(SDNS)有效。然而,关于RTX治疗后复发的危险因素和长期预后的数据不足。
2007年11月至2013年12月,我们对难治性SDNS患者给予单剂量RTX,并继续使用免疫抑制剂。分析早期复发和长期预后的危险因素。
纳入81例患者,观察期为13 - 90个月。76例患者(94%)停用了类固醇。B细胞耗竭的中位持续时间为160天,50%的无复发生存期为482天。在多变量分析中,只有类固醇抵抗型肾病综合征(SRNS)病史是具有统计学意义的危险因素(风险比,2.44;p = 0.048)。无SRNS病史患者的50%无复发生存期为615天,长于有SRNS病史患者(393天)(p = 0.005)[校正后]。51例患者(63%)因复发接受了额外的RTX治疗。在最后一次观察时,使用钙调神经磷酸酶抑制剂的患者从89%降至23%,12例患者(15%)停用了免疫抑制剂。
对于SDNS患者,RTX治疗后继以免疫抑制剂是一种有效的选择,尽管SRNS病史是早期复发的危险因素。