ESPN 51th Annual Meeting

ESPN 2018


 
ESPN survey on current practice regarding recurrent focal-segmental glomerulosclerosis after pediatric kidney transplantation.
ANTONIA BOUTS 1 FLOOR VELTKAMP 1 BURKHARD TÖNSHOFF 2 MARINA VIVARELLI 3

1- DEPARTMENT OF PEDIATRIC NEPHROLOGY, EMMA CHILDRENS HOSPITAL, AMC, AMSTERDAM, THE NETHERLANDS
2- DEPARTMENT OF PEDIATRICS I, UNIVERSITY CHILDRENS HOSPITAL HEIDELBERG, HEIDELBERG, GERMANY
3- DIVISION OF NEPHROLOGY AND DIALYSIS, BAMBINO GESU CHILDRENS HOSPITAL AND RESEARCH INSTITUTE, ROME, ITALY
 
Introduction:

Primary focal-segmental glomerulosclerosis (FSGS) is an important cause of end-stage renal failure in pediatric patients. The post-transplantation (post-tx) recurrence risk of non-genetic FSGS is high and is associated with an increased risk of early graft loss. No clear treatment guidelines for FSGS recurrence exist.

Material and methods:

We investigated current practice through a web-based survey on behalf of the working groups “Idiopathic Nephrotic Syndrome” and “Renal Transplantation” of the European Society of Pediatric Nephrology (ESPN).

Results:

62 (pediatric) nephrologists (59 centers) from 31 countries responded to the survey. The total number of transplanted FSGS patients was 1262 (median 0.76 cases/year/center), with 241 recurrences post-tx (median 0.24 cases/year/center). Nephrectomy was always performed before and/or during transplantation in 22 of 59 (37%) centers, never in 23 (39%) and on clinical indication in 10 (17%). 29 (49%) centers treated their patients before transplantation to prevent recurrence post-tx: 10 using plasmapheresis/(double)filtration (PF), 4 rituximab, 9 combination of PF/immunoadsorption(IA)/rituximab, 2 cyclosporine, 4 unknown. The initial immunosuppressive regimen for FSGS without proven mutation consisted of aIL2R+corticosteroids+CNI (tacrolimus/cyclosporine) + mycophenolate mofetil in 36 (61%) of the centers; in case of known mutation, 37 (63%) centers used this regimen. The reported median (range) percentage of recurrence post-tx <1 week was 33% (0-100%). Treatment of post-tx recurrence was as follows: 1) PF and/or IA: 53 centers (90%), 2) Rituximab: 44 centers (75%), 3) ACE/ARB: 39 centers (66%), 4) Corticosteroids: 33 centers (56%), 5) switch immunosuppressive regimen: 14 centers (24%). Complete remission in >50% of the patients was reported in 21 (41%) centers and in <10% in 9 (18%).

Conclusions:

This survey showed that recurrence of FSGS within 1 week after transplantation is common (33%). Complete remission in >50% of the patients was achieved in 41% of the centers. Future analysis will focus on treatment and predisposing factors, including biopsy findings and genetic mutation results.