Department of Pediatrics, Divisions of Nephrology and Critical Care, Duke University Medical Center, Durham, NC, 27710, USA.
Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois and Children's Hospital Seattle, Washington, USA.
Pediatr Nephrol. 2018 Oct;33(10):1773-1780. doi: 10.1007/s00467-018-3994-3. Epub 2018 Jul 7.
Steroid-resistant nephrotic syndrome (SRNS) due to focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is a leading cause of end-stage kidney disease in children. Recurrence of primary disease following transplantation is a major cause of allograft loss. The clinical determinants of disease recurrence are not completely known. Our objectives were to determine risk factors for recurrence of FSGS/MCD following kidney transplantation and factors that predict response to immunosuppression following recurrence.
Multicenter study of pediatric patients with kidney transplants performed for ESKD due to SRNS between 1/2006 and 12/2015. Demographics, clinical course, and biopsy data were collected. Patients with primary-SRNS (PSRNS) were defined as those initially resistant to corticosteroid therapy at diagnosis, and patients with late-SRNS (LSRNS) as those initially responsive to steroids who subsequently developed steroid resistance. We performed logistic regression to determine risk factors associated with nephrotic syndrome (NS) recurrence.
We analyzed 158 patients; 64 (41%) had recurrence of NS in their renal allograft. Disease recurrence occurred in 78% of patients with LSRNS compared to 39% of those with PSRNS. Patients with MCD on initial native kidney biopsy had a 76% recurrence rate compared with a 40% recurrence rate in those with FSGS. Multivariable analysis showed that MCD histology (OR; 95% CI 5.6; 1.3-23.7) compared to FSGS predicted disease recurrence.
Pediatric patients with MCD and LSRNS are at higher risk of disease recurrence following kidney transplantation. These findings may be useful for designing studies to test strategies for preventing recurrence.
由局灶节段性肾小球硬化症(FSGS)和微小病变病(MCD)引起的激素耐药性肾病综合征(SRNS)是儿童终末期肾病的主要原因。移植后原发性疾病的复发是导致移植物丢失的主要原因。疾病复发的临床决定因素尚不完全清楚。我们的目的是确定儿童肾移植后 FSGS/MCD 复发的危险因素,以及预测复发后免疫抑制反应的因素。
对 2006 年 1 月至 2015 年 12 月间因 SRNS 导致终末期肾病而接受肾移植的儿科患者进行了多中心研究。收集了人口统计学、临床过程和活检数据。将原发性 SRNS(PSRNS)定义为在诊断时最初对皮质类固醇治疗耐药的患者,将迟发性 SRNS(LSRNS)定义为最初对类固醇有反应但随后出现类固醇耐药的患者。我们进行了逻辑回归分析,以确定与肾病综合征(NS)复发相关的危险因素。
我们分析了 158 例患者;64 例(41%)的肾移植后出现 NS 复发。LSRNS 患者中,78%的患者出现疾病复发,而 PSRNS 患者中,这一比例为 39%。在初始原发性肾脏活检中,MCD 患者的复发率为 76%,而 FSGS 患者的复发率为 40%。多变量分析显示,与 FSGS 相比,MCD 组织学(OR;95%CI 5.6;1.3-23.7)预测疾病复发。
患有 MCD 和 LSRNS 的儿科患者在肾移植后发生疾病复发的风险更高。这些发现可能有助于设计研究,以测试预防复发的策略。