Focal segmental glomerular sclerosis (FSGS) is the most common glomerular disease leading to end-stage renal disease (ESRD) in children. FSGS frequently recurs after kidney transplant and may cause graft failure. Therapeutic plasma exchange (TPE), rituximab and steroids have been used to treat post-transplant FSGS recurrence, but limited data is available to support their combined efficacy.

We conducted a retrospective cohort study of all kidney transplant recipients at a single pediatric center between 2007 and 2018 to determine frequency of FSGS and early recurrence of nephrotic syndrome within 4 days of transplant. Primary outcome was graft survival and secondary outcome was remission of nephrotic syndrome.

FSGS was the indication for renal transplant in 11% of cases(n=29). Of these, 24% of patients had early FSGS recurrence after transplant(n=7). Treatment was at the discretion of the treating physician, but generally included TPE, rituximab and steroids.  Five responded; the other two returned to chronic dialysis. 22 patients without recurrence experienced 100% 1-year graft survival. For all FSGS recipients, including those with recurrence, 3-year graft survival was 94.4%. 5/7(71%) of recipients with recurrence achieved partial or full remission of nephrotic syndrome following treatment.

This study, the largest to uniformly employ the combination of TPE, rituximab and high-dose steroids, supports the efficacy of this treatment for pediatric patients with FSGS recurrence.