Recurrent focal segmental glomerulosclerosis in grafts treated with plasmaexchange and increased immunosuppression

We report on three children with severe, recurrent focal segmental glomerul oscerosis (FSGS) in their first allografts, treated with methylprednisolone , plasma exchange and cyclophosphamide. This protocol is based on a previou s publication showing its successful use in three children. Our patients we re 2 girls and 1 boy, aged 14.5, 14.6 and 13.2 years, respectively, at tran splant. Concomitant immunosuppression included cyclosporin A and prednisolo ne. Recurrence occurred in all three patients within 24 h, and specific tre atment was commenced within 48 h. All patients developed anuria and were di alysed. The boy stopped dialysis after 4 weeks, and has stable chronic rena l failure (CRF) and no proteinuria 3 years later. One girl required dialysi s for 4 months, and 3 years later has CRF with non-nephrotic range proteinu ria. The other girl remained dialysis-dependent and died from septic compli cations. We conclude that even anuric patients treated with this protocol m ay have an improvement in renal function and reduction of proteinuria, whic h can last for over 3 years. However, treatment may need to be prolonged an d carries the substantial risks of heavy immunosuppression.