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Minerva Pediatrica 2016 Ottobre;68(5):348-54

lingua: Inglese

Recurrence of nephrotic proteinuria in children with focal segmental glomerulosclerosis: early treatment with plasmapheresis and immunoadsorption should be associated with better prognosis

Filip FENCL 1, Karel VONDRÁK 1, Tomáš ROSÍK 1, Jakub ZIEG 1, Mária CHADIMOVÁ 2, Jaromír HÁČEK 2, Jiří DUŠEK 1, Tomáš SEEMAN 3

1 Department of Pediatrics, Second Faculty of Medicine, Charles University and Motol in Prague and University Hospital, Prague, Czech Republic; 2 Department of Pathology and Molecular Medicine, Second Faculty of Medicine, Charles University and Motol in Prague and University Hospital, Prague, Czech Republic; 3 Biomedical Centre, Faculty of Medicine in Plzen, Charles University, Plzen, Czech Republic


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BACKGROUND: Primary focal segmental glomerulosclerosis (FSGS) is a glomerular disease, characterized by progressive renal function deterioration, nephrotic proteinuria, and risk of chronic renal failure. We present long-term results of 5 patients with primary FSGS and recurrence of nephrotic proteinuria after renal transplantation treated with plasma exchange (PE) and immunoadsorption (IA).
METHODS: We retrospectively investigated the relationship between the delay in initiation of the therapy and treatment outcomes, particularly achievement of remission of proteinuria.
RESULTS: Remission occurred in all three patients who started PE/IA in interval 3-7 days after diagnosis of recurrence of FSGS. Remission was achieved after 3-4 weeks in two patients with 3 days of delay to the start of PE. The third patient (PE started with 7 days of delay) reached complete remission after 6 months of PE/IA treatment. All these patients had remission sustainable for a long time. The remaining two patients with 14 and 406 days of delay to PE treatment did not achieve remission sustainable for a long time. The two patients who did not achieve remission developed end-stage renal disease with graft loss (1 and 6.7 years after transplantation). Patients who achieved remission of proteinuria during PE/IA treatment have still functioning grafts (2.8, 9.7 and 3.8 years after renal transplantation). All these patients are still treated with PE/IA.
CONCLUSIONS: The present 5 cases suggest that if recurrence of FSGS occurs, the probability of achieving remission is dependent on the early initiation of PE/IA therapy. Therefore, we suggest that PE/IA treatment might be started as soon as possible after recurrence of FSGS.

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filip.fencl@lfmotol.cuni.cz