Background: Up to 60% of pediatric renal transplant recipients with end-stage renal disease due to primary focal and segmental glomerulosclerosis (FSGS) may develop recurrent disease. Such recurrence is associated with poor prognosis if no remission is achieved. We report a single center experience with a protocol based on plasmaphereis and increased immunosuppression that resulted in a high long-lived remission rates.
Methods: This retrospective cohort study included consecutive pediatric renal transplant patients with recurrent FSGS treated with a standardized protocol using plasmapheresis and cyclophosphamide to supplement usual post-transplant immunosuppression with calcineurin inhibitors and steroids. Relapse was defined as urinary protein/creatinine ratio >1.0g/g and remission as <0.5g/g.
Results: 17 patients with FSGS recurrence post-transplant were treated. All had therapy-resistant FSGS in native kidneys and had been on dialysis from 4-10 years. Of the 17, one died perioperatively from a pulmonary thromboembolism. 12 others achieved a complete remission within 3 months of treatment for FSGS recurrence. After a median follow-up period of 4 years, there were no recurrences of significant proteinuria.  
Conclusion: The addition of plasmapheresis and cyclophosphamide to a calcineurin- and steroid-based immunosuppression regime could be an successful in inducing high remission rates with recurrent FSGS.  Prospective trials are needed to evaluate further the efficacy of increased immunosuppression along with plasmapheresis  in this setting.
Keywords: Focal and segmental glomerulosclerosis, renal transplantation, recurrence, plasmapheresis, cyclosphosphamide, pediatric