PMCID: PMC6129203 DOI: 10.1007/s00467-018-3994-3
BACKGROUND AND OBJECTIVES: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
Pediatric nephrology (Berlin, Germany)
Adolescent; Adult; Biopsy; Child; Child, Preschool; Drug Resistance; Female; Glomerulosclerosis, Focal Segmental; Glucocorticoids; Graft Rejection; Humans; Infant; Infant, Newborn; Kidney; Kidney Transplantation; Male; Nephrosis, Lipoid; Nephrotic Syndrome; Preoperative Period; Prognosis; Recurrence; Retrospective Studies; Risk Factors; Young Adult
Focal segmental glomerulosclerosis; Immunosuppression; Lipoid; Nephrosis; Nephrotic syndrome; Transplantation
Pelletier, J. H., Kumar, K. R., Engen, R., Bensimhon, A., Varner, J. D., Rheault, M. N., Srivastava, T., Straatmann, C., Silva, C., Davis, T. K., Wenderfer, S. E., Gibson, K., Selewski, D., Barcia, J., Weng, P., Licht, C., Jawa, N., Kallash, M., Foreman, J. W., Wigfall, D. R., Chua, A. N., Chambers, E., Hornik, C. P., Brewer, E. D., Nagaraj, S. K., Greenbaum, L. A., Gbadegesin, R. A. Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings. Pediatric nephrology (Berlin, Germany) 33, 1773-1780 (2018).