A Case of Early Recurrence of Focal Segmental Glomerulosclerosis after Renal Transplant - EAP 2017 Congress and MasterCourse, October 12-15, 2017, Ljubljana, Slovenia

ADRIANA MONICA BUNGARDI ¹ Aurel Bizo ^1,2 Cornel Aldea ¹ Bogdan Bulata ¹ Mihai Lucan ^3,4 Dan Gheban ⁵ Dan Delean ¹

¹Nephrology and Dialysis Department, Emergency Clinic Hospital for Children
²Pediatric Nephrology Department, University of Medicine and Pharmacy “Iuliu Hatieganu”
³Renal Transplant Department, Urology and Renal Transplant Institute
⁴Urology Department, University of Medicine and Pharmacy “Iuliu Hatieganu”
⁵Department of Anatomical Pathology, Emergency Clinic Hospital for Children

Introduction: Primary focal segmental glomerulosclerosis (FSGS) is one of the most severe glomerular diseases, with multifactorial and often undefined pathogenesis. It presents with proteinuria, usually of nephrotic range, and progression to end-stage renal disease (ESRD) is frequent.

FSGS is the initial renal disease in approximately one-tenth of pediatric patients receiving a renal allograft. In the first renal transplantation, the nephrotic syndrome and progressive glomerular sclerosis recur in 30 to 40% of patients, which negatively impact graft survival.

Case-report: We present the case of a 16-year-old Caucasian girl with primary FSGS, which debuted as a pure nephrotic syndrome, refractory to corticosteroids and other immunosuppressive agents. It slowly progressed to ESRD, for which she necessitated chronic hemodialysis until the possibility of transplantation. After the renal transplant from a deceased donor, which occurred 4 years after the onset of FSGS, there were immediate complications: a lymphocele and a subcapsular hematoma, leading to acute tubular necrosis. Due to the urological reintervention, plasmapheresis and immunosuppressive treatment, the renal function rapidly improved. 2 months after the transplantation, the laboratory tests revealed impaired renal function with nephrotic range proteinuria. It was interpreted as an early recurrence of FSGS, uninfluenced by medical therapy or plasmapheresis, requiring allograft removal and resumption of chronic hemodialysis.

Conclusion: Compared to other renal diseases, the incidence of delayed graft function is higher and graft survival is lower in children with FSGS. At the present time, most researchers consider FSGS as a podocyte disease, but the pathogenesis of recurrent FSGS is far from being established.

In spite of the risk of recurrence and the therapy for FSGS in renal graft being largely empirical, patients with FSGS should not be excluded from transplantation.