Stratifying risk of post transplant recurrence and transplant failure in children with steroid resistant nephrotic syndrome: A study from the Australian and New Zealand paediatric nephrology association

Abstract

Aim: To describe the impact of initial steroid sensitivity on risk of disease recurrence and transplant failure for children following kidney transplantation for steroid resistant nephrotic syndrome (SRNS). Background: Scarce prior data suggest that children transplanted for SRNS who were steroid responsive at their initial presentation have an increased risk of recurrence post transplantation. There are no data informing the impact of initial steroid sensitivity on transplant survival. Methods: Children who received a kidney transplant between 2000 and 2019 for SRNS in 7 paediatric centres were included. Clinical data were gathered from chart reviews and transplant data were provided from ANZDATA following ethics approval. Transplant survival was estimated using the Kaplan-Meier estimator, and the association between variables and graft survival estimated by Cox modelling. Results: There were 56 children, 32 (57%) male, average age at transplant 11 years (SD 4) and 36 (65%) Caucasian. At presentation, 19/56 (34%) were steroid sensitive before developing SRNS. Of those steroid sensitive initially, 18/19 (95%) had recurrence post-transplant, 9/18 (50%) achieved full remission, although 3 of these had further recurrences. In those not steroid sensitive initially, 14/37 (38%) had recurrence post-transplant, 7/14 (50%) achieved full remission although one had further recurrences. Overall, 5-year transplant survival was 72% (95%CI = 60-86%) and initial steroid sensitivity was not significantly associated with transplant loss (p = 0.07). Conclusions: Initial steroid sensitivity is strongly associated with SRNS recurrence. Transplant survival for children with SRNS is poor. Further research in other cohorts is needed to assess if initial steroid sensitivity is associated with poorer graft outcomes.L6339279002021-01-20 <br />