Division of Nephrology, Department of Medicine, Washington University School of Medicine, St. Louis, MO.
Transplant Epidemiology Research Collaboration (TERC), Institute of Public Health, Washington University School of Medicine, St. Louis, MO.
Transplantation. 2019 Jan;103(1):202-209. doi: 10.1097/TP.0000000000002320.
Treatment of focal segmental glomerular sclerosis (FSGS) after kidney transplantation is challenging with unpredictable outcomes. The objective was to investigate the use of adrenocorticotropic hormone (ACTH) analogue gel in kidney transplant recipients with de novo or recurrent FSGS resistant to therapeutic plasma exchange (TPE) and/or rituximab.
We performed a retrospective review of cases of de novo or recurrent resistant FSGS at 2 large US transplant centers between April 2012 and December 2016. Proteinuria was measured by urine protein to creatinine ratio.
We identified 20 cases of posttransplant recurrent and de novo FSGS resistant to conventional therapy with TPE and rituximab. Mean ± SD age was 49 ± 15.5 years, 14 (70%) were male, 13 (65%) were whites, and 8 (38%) had previous kidney transplants. Median (interquartile range) of recurrent and de novo FSGS was 3 (0.75-7.5) months posttransplant. The majority of patients, 15 (75%), received TPE as a treatment at the time of diagnosis and 10 (50%) received rituximab, which was started before the use of ACTH gel. There was a significant improvement of urine protein to creatinine ratio from a mean ± SD of 8.6 ± 7.6 g/g before ACTH gel to 3.3 ± 2.3 g/g after the use of ACTH gel (P = 0.004). Ten (50%) patients achieved complete or partial remission.
Although, the response varied among the recipients, ACTH gel might be an effective therapy for posttransplant resistant FSGS cases that fail to respond to TPE and rituximab.
肾移植后局灶节段性肾小球硬化症(FSGS)的治疗具有挑战性,且结果难以预测。本研究旨在探讨促肾上腺皮质激素(ACTH)类似物凝胶在治疗对治疗性血浆置换(TPE)和/或利妥昔单抗治疗抵抗的新发或复发性 FSGS 肾移植受者中的应用。
我们对 2012 年 4 月至 2016 年 12 月期间美国 2 个大型移植中心的新发或复发性 FSGS 病例进行了回顾性研究。蛋白尿通过尿蛋白与肌酐比值来衡量。
我们共发现 20 例对 TPE 和利妥昔单抗常规治疗抵抗的移植后复发和新发 FSGS 病例。平均年龄为 49±15.5 岁,14 例(70%)为男性,13 例(65%)为白人,8 例(38%)有既往肾移植史。新发和复发性 FSGS 的中位数(四分位距)为移植后 3(0.75-7.5)个月。大多数患者(75%)在诊断时接受了 TPE 治疗,10 例(50%)在使用 ACTH 凝胶前接受了利妥昔单抗治疗。在使用 ACTH 凝胶后,尿蛋白与肌酐比值从使用前的 8.6±7.6 g/g 显著改善至 3.3±2.3 g/g(P=0.004)。10 例(50%)患者达到完全或部分缓解。
尽管患者之间的反应存在差异,但 ACTH 凝胶可能是治疗对 TPE 和利妥昔单抗治疗抵抗的移植后 FSGS 病例的有效治疗方法。