561 - Successful treatment of recurrent focal segmental glomerulosclerosis (FSGS) in pediatric kidney transplant patients

Publication Number: 4549.561

Corinna K. Victor, Louisiana State University School of Medicine in New Orleans, New Orleans, LA, United States; Diego Aviles, LSU Health Sciences Center, Metairie, LA, United States; Kiara A. Tulla, Tulane School of Medicine, New Orleans, LA, United States; Caroline E. Straatmann, LSU Health and Manning Family Children's, New Orleans, LA, United States

Background: FSGS recurs at a variable rate in pediatrics and can lead to graft loss. Podocyte injury may be mediated by a circulating plasma factor. Treatment and response vary by center.

Objective: We examined the efficacy of early, prolonged plasmapheresis (PP) in pediatric transplant recipients with recurrent FSGS.

Design/Methods: This is a retrospective review of our center's 41 patients who underwent kidney transplantation for FSGS between 1995 and 2024. Recurrence was defined by progressive postoperative increase in proteinuria. Renal biopsy, if performed, showed only foot process effacement. 19 of 41 patients (46%) had FSGS recurrence. Of those who recurred, 8 received a living donor graft. 10 had bilateral nephrectomies prior to transplant. 16 patients received standard steroid-based immunosuppression, and the remaining 3 patients were converted to steroid-based upon FSGS recurrence. The average time to recurrence was 2.7 days (ranging from 1 to 9 days), excluding one patient who recurred 28 days after transplant. In the patients who recurred immediately postoperatively, time to initiation of PP ranged from postoperative day 2 to 15. Peak urine protein to creatinine ratio (UPCR) prior to starting PP ranged from 1.7 to 207 mg/mg. All patients received PP daily for 4 to 66 days and were gradually weaned off by increasing PP intervals, guided by daily morning UPCR. PP was discontinued when UPCR was consistently less than 0.5 mg/mg.

Results: The total course of PP for all 19 patients ranged from 4 to 37 weeks. Eight patients also received additional immunosuppressive agents. 17 of 19 patients (89%) achieved complete remission. Two patients did not respond and had graft loss. Two patients with the shortest initial course of PP (less than 6 weeks) had a second FSGS recurrence within weeks of discontinuing PP and both remitted again after an additional 3 to 6 weeks of PP. Median observation period was 4.4 years (1.8 to 17 years). All patients maintained complete remission for the duration of pediatric follow up. One patient currently has ESRD secondary to severe transplant rejection, but UPCR remains less than 1 mg/mg. The remaining patients currently have serum creatinine ranging from 0.7 to 3.9 mg/dL and UPCR 0.07 to 0.9 mg/mg, except for one patient with UPCR of 3.2 mg/mg due to chronic allograft nephropathy.

Conclusion(s): PP was successful in achieving full remission of FSGS recurrence in almost 90% of our patients over the last three decades. Early diagnosis of recurrence, prompt initiation of PP, and an intensive and prolonged course of PP may be key factors to successful treatment and prevention of graft loss.

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