Clinical Characteristics and Favorable Treatment Responses of Recurrent Focal Segmental Glomerulosclerosis Or steroid-resistant Nephrotic Syndrome in Children After Kidney Transplantation

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 2024 Jul 13

PMID 39001911

Authors

Vikas R Dharnidharka

Rebecca R Scobell

Mahmoud Kallash

Amy J Goodwin Davies

Nicole Marchesani

Mitchell G Maltenfort

Leslie Walther

Megan Kelton

Margret Bock

Eliza Blanchette

Hillarey K Stone

Caroline Gluck

Frank Hullekes

Leonardo V Riella

William E Smoyer

Mark Mitsnefes

Bradley P Dixon

Joseph T Flynn

Michael J G Somers

Christopher B Forrest

Susan Furth

Michelle R Denburg

Affiliations

Soon will be listed here.

Abstract

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.

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