背景:肾移植后局部节段肾小球硬化(FSGS)或类固醇抗性肾病综合征(SRNS)的复发导致了显著的发病率和潜在的早期同种异体移植丢失。然而,迄今为止,报告率,危险因素和治疗结果差异很大.
方法:我们将计算表型应用于来自美国7个大型儿科卫生系统的电子健康记录数据的多中心集合,为了确定复发率,危险因素,和治疗结果。我们通过图表审查来完善数据收集。
结果:来自>700万患者,我们比较了原发性FSGS/SRNS患儿,这些患儿在2009年至2020年期间接受了肾脏移植,出现复发(n=67/165;40.6%)或未复发(n=98/165).在复发组中,移植时的血清白蛋白水平显着降低,而受体HLADR7的存在显着升高。移植后36个月,58.2%完全缓解,17.9%部分缓解.移植后6年,复发后无缓解与同种异体移植物丢失的风险随时间增加相关(p<0.0001),但是任何缓解都显示出与无复发者相似的同种异体移植存活率和功能下降。由于治疗是以非随机方式使用的,使用样条曲线和多变量非线性分析,完全+部分缓解的机会显着增加血浆置换的疗程,CTLA4-Ig剂量或LDL-单采术。仅使用抗CD20,CTLA4-Ig药物治疗,或LDL-单采治疗与完全缓解相关.排除25例突变患者并没有显着改变我们的结果。
结论:我们的当代高风险队列比大多数以前的报告有更高的良好反应率,来自代理的组合。
BACKGROUND: Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely.
METHODS: We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review.
RESULTS: From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results.
CONCLUSIONS: Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents.