Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
University of Wisconsin Madison, Madison, Wisconsin, USA.
Pediatr Transplant. 2022 Sep;26(6):e14307. doi: 10.1111/petr.14307. Epub 2022 May 19.
FSGS is a common indication for kidney transplant with a high-risk of posttransplant recurrence.
In this review, we summarize current knowledge about FSGS recurrence after kidney transplantation, including epidemiology, pretransplant planning, posttransplant management, and investigational treatments.
FSGS recurs in 14%-60% of first transplants, likely associated with a circulating permeability factor. Pretransplant counseling regarding recurrence is critical, and patients with FSGS should undergo pretransplant genetic screening. Rapid progression to ESKD, initial steroid responsiveness, younger age at diagnosis, race/ethnicity, and mesangial hypercellularity or minimal change histology on native biopsy may be associated with recurrence. Living donation is not contraindicated but does not result in improved graft survival relative to deceased donation. Pretransplant nephrectomy may be performed for a variety of reasons, but does not decrease recurrence. Pretransplant therapy with rituximab and/or PE is understudied but not clearly effective at preventing recurrence. Patients with FSGS typically present early with rapid-onset severe proteinuria. Diagnosis can be confirmed by biopsy showing foot process effacement; typical FSGS lesions are not seen on light microscopy in the early stages. There is no established effective treatment for recurrent FSGS, but renin-angiotensin-aldosterone system inhibition and extracorporeal therapies, including PE and IA, are most commonly used. Adjunct or alternative therapies may include rituximab, lipopheresis, and cyclosporine.
FSGS 是肾移植的常见适应证，其移植后复发的风险较高。
在本综述中，我们总结了 FSGS 肾移植后复发的相关知识，包括流行病学、移植前规划、移植后管理和试验性治疗。
首次移植后 FSGS 的复发率为 14%-60%，可能与循环通透性因子有关。移植前对复发的咨询至关重要，FSGS 患者应进行移植前基因筛查。快速进展为终末期肾病、初始激素反应性、诊断时年龄较小、种族/民族、系膜细胞增生或原发性活检的微小病变组织学可能与复发相关。活体供肾并不被禁止，但与尸肾供体相比并不能改善移植物存活率。为多种原因可进行移植前肾切除术，但不能降低复发率。利妥昔单抗和/或血浆置换（PE）的移植前治疗研究较少，但似乎不能有效预防复发。FSGS 患者通常早期出现快速发作的严重蛋白尿。通过活检显示足细胞足突消失可确诊；在早期阶段，光镜下看不到典型的 FSGS 病变。目前，尚无针对复发性 FSGS 的有效治疗方法，但肾素-血管紧张素-醛固酮系统抑制和体外治疗，包括 PE 和 IA，是最常用的方法。辅助或替代治疗可能包括利妥昔单抗、脂蛋白吸附和环孢素。
