Recurrence of focal segmental glomerulosclerosis after kidney transplantation: strategies and outcome

Abstract

Purpose of review: Steroid-resistant nephrotic syndrome/focal segmental glomerulonephritis (FSGS) is the primary renal disease in approximately 10% of pediatric patients receiving a renal allograft. Risk factors for recurrence are a chronological age between 6 and 15 years at onset of the nephrotic syndrome and a rapid progression of the disease in the native kidneys leading to end-stage renal disease in less than 3 years. With rapid recurrence of FSGS and loss of the allograft, further renal transplants also carry a high likelihood of recurrence of nephrotic syndrome.

Recent findings: Different pathogenic factors have been discussed for the recurrence of proteinuria/FSGS in the transplanted kidney, especially the involvement of a proteinuric circulating factor. Treatment strategies are divided into two phases: induction of remission by plasma exchanges combined with high-dose intravenous or oral cyclosporine A; stabilization of remission by cyclophosphamide or rituximab, which showed promising results in several case reports.

Summary: No controlled studies have been performed yet to address the management of recurrent FSGS posttransplant. Complications related to the high-degree immunosuppression are not rare and should be regularly investigated. Therefore, the benefit: risk ratio for all immunosuppressive treatment strategies should be carefully evaluated for each individual patient.