Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation

• Published in: Pediatric nephrology (Berlin, West) West), 2024-11, Vol.39 (11), p.3317-3331
• Main Authors: Dharnidharka, Vikas R., Scobell, Rebecca R., Kallash, Mahmoud, Davies, Amy J. Goodwin, Marchesani, Nicole, Maltenfort, Mitchell G., Walther, Leslie, Kelton, Megan, Bock, Margret, Blanchette, Eliza, Stone, Hillarey K., Gluck, Caroline, Hullekes, Frank, Riella, Leonardo V., Smoyer, William E., Mitsnefes, Mark, Dixon, Bradley P., Flynn, Joseph T., Somers, Michael J. G., Forrest, Christopher B., Furth, Susan, Denburg, Michelle R.
• Format: Article
• Language: English
• Subjects: Adolescent; Allografts; Apheresis; CD20 antigen; Child; Clinical outcomes; CTLA-4 protein; Data collection; Electronic medical records; Female; Glomerulosclerosis, Focal Segmental - diagnosis; Glomerulosclerosis, Focal Segmental - drug therapy; Glomerulosclerosis, Focal Segmental - surgery; Graft Survival - drug effects; Humans; Immunoglobulins; Immunosuppressive Agents - adverse effects; Immunosuppressive Agents - therapeutic use; Kidney diseases; Kidney transplantation; Kidney Transplantation - adverse effects; Kidney transplants; Low density lipoprotein; Male; Medicine; Medicine & Public Health; Morbidity; Nephrology; Nephrotic syndrome; Nephrotic Syndrome - diagnosis; Nephrotic Syndrome - drug therapy; Nephrotic Syndrome - surgery; Original Article; Patients; Pediatrics; Phenotypes; Plasmapheresis; Recurrence; Remission; Remission Induction; Retrospective Studies; Risk Factors; Steroids; Treatment Outcome; United States - epidemiology; Urology; What’s New in Renal Transplantation
• ISSN: 0931-041X; 1432-198X; 1432-198X
• DOI: 10.1007/s00467-024-06452-z

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