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Indexed/Abstracted in: CAB, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 0,532
Online ISSN 1827-1715
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
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.