Abstract: TH-PO144

Initial Response to Corticosteroid Therapy and Native Kidney Biopsy Findings Predict Disease Recurrence Following Kidney Transplantation in Childhood Nephrotic Syndrome

Session Information

Category: Glomerular

  • 1005 Clinical Glomerular Disorders

Authors

  • Bensimhon, Adam R, Duke University Medical Center, Durham, North Carolina, United States
  • Engen, Rachel M., Duke University Medical Center, Durham, North Carolina, United States
  • Gibson, Keisha L., Duke University Medical Center, Durham, North Carolina, United States
  • Licht, Christoph, Duke University Medical Center, Durham, North Carolina, United States
  • Selewski, David T., Duke University Medical Center, Durham, North Carolina, United States
  • Greenbaum, Larry A., Duke University Medical Center, Durham, North Carolina, United States
  • Gbadegesin, Rasheed A., Duke University Medical Center, Durham, North Carolina, United States
  • Pelletier, Jonathan H., Duke University Medical Center, Durham, North Carolina, United States
  • Kumar, Karan, Duke University Medical Center, Durham, North Carolina, United States
  • Rheault, Michelle N., Duke University Medical Center, Durham, North Carolina, United States
  • Srivastava, Tarak, Duke University Medical Center, Durham, North Carolina, United States
  • Straatmann, Caroline E., Duke University Medical Center, Durham, North Carolina, United States
  • Davis, Thomas K., Duke University Medical Center, Durham, North Carolina, United States
  • D'Alessandri-Silva, Cynthia J., Duke University Medical Center, Durham, North Carolina, United States
  • Wenderfer, Scott E., Duke University Medical Center, Durham, North Carolina, United States
Background

Steroid resistant nephrotic syndrome (SRNS) is a leading cause of ESKD in children. Disease recurrence following kidney transplantation is the single most important cause of renal allograft loss in SRNS. Previous studies have not consistently identified risk factors associated with recurrence of disease. This study aims to determine predictors of disease recurrence in children with SRNS after renal transplantation.

Methods

A 10 year multicenter review of kidney transplants performed for SRNS in MWPNC participating centers. Data were collected on patients' demographics, clinical course, and biopsy findings. Patients with primary SRNS (PSRNS) were defined as those initially resistant to steroid therapy at diagnosis, and patients with late SRNS (LSRNS) were defined as those initially responsive who subsequently developed resistance. Groups were compared by chi-square tests or Fisher's exact test, and multivariate regression analysis was used to identify predictors of recurrence.

Results

We identified 116 patients (67 with PSRNS, 17 with LSRNS, and 32 with genetic SRNS/undetermined pattern of response). Disease recurrence occurred in 35.8% of patients with PSRNS compared to 70.6% of those with LSRNS (p < 0.001). Age at diagnosis and ESKD, sex, race, and ethnicity were similar in patients with and without recurrence. Patients with minimal change disease histology (MCD) on initial kidney biopsy had an 80% recurrence rate compared with a 31% recurrence rate in those with focal segmental glomerulosclerosis (FSGS) (p = 0.005). Unadjusted multivariate analysis identified MCD (OR 8.9; 95% CI 1.8-45.3, p = 0.008) and LSRNS (OR 4.3; 95% CI, 1.4 to 13.7, p = 0.013) as predictors of disease recurrence, but in adjusted analysis, only LSRNS predicted disease recurrence (OR 4.3; 95% CI 1.1-16.1, p = 0.032).

Conclusion

Pediatric patients with LSRNS and MCD histology are at significantly higher risk of disease recurrence following kidney transplantation. These findings may be useful for designing studies to test strategies for preventing recurrence.

Funding

  • NIDDK Support