Post-transplant recurrence of steroid resistant nephrotic syndrome in children: the Italian experience

William Morello,Massimo Cardillo,Gianluca Caridi,Luca Dello Strologo,Luisa Murer,Giovanni Montini,Isabella Guzzo,Michela Cioni,Licia Peruzzi,Enrico Cocchi,Sara Testa,Rosanna Torelli,Giuseppe Puccio,Sairaj Puvinathan,Elisa Benetti,Luciana Ghio,Gian Marco Ghiggeri

doi:10.1007/s40620-019-00660-9

Abstract

BackgroundSteroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss.MethodsWe identified all children with SRNS who underwent renal transplantation in Italy, between 2005 and 2017. Data were retrospectively collected for the presence of a causative gene mutation, sex, histology, duration of pre-transplant dialysis, age at onset and transplant, HLA matching, recurrence, therapy for recurrence, and graft survival.Results101 patients underwent a first and 22 a second renal transplant. After a median follow-up of 58.5 months, the disease recurred on the first renal transplant in 53.3% of patients with a non-genetic and none with a genetic SRNS. Age at transplant > 9 years and the presence of at least one HLA-AB match were independent risk factors for recurrence. Duration of dialysis was longer in children with relapse, but did not reach statistical significance. Overall, 24% of patients lost the first graft, with recurrence representing the commonest cause. Among 22 patients who underwent a second transplant, 5 suffered of SRNS recurrence. SRNS relapsed in 5/9 (55%) patients with disease recurrence in their first transplant and 2 of them lost the second graft.ConclusionsAbsence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. A prolonged time spent on dialysis before transplantation has no protective effect on the risk of relapse and should not be encouraged. Retransplantation represents a second chance after graft loss for recurrence.

Highlights

Steroid resistant nephrotic syndrome (SRNS) is the most common acquired cause of end stage renal failure (ESRD) requiring transplantation in children
A total of 728 (618 deceased and 110 living donors) renal grafts were performed at the 5 Italians pediatric transplant centres, of whom 123 in patients with ESRD secondary to SRNS. 101 patients received a first renal allograft and 22 a second renal transplant (12 failures of the original cohort and 10 failures of a first transplant that occurred before the study period)
Renal histology was consistent with focal segmental glomerulosclerosis (FSGS) in 85 cases, minimal change disease (MCD) in 14 and diffuse mesangial sclerosis (DMS) in 2

Summary

Introduction

Steroid resistant nephrotic syndrome (SRNS) is the most common acquired cause of end stage renal failure (ESRD) requiring transplantation in children. In up to 50% of patients, SRNS relapses after transplantation and disease recurrence is a major cause of graft loss [4,5,6]. Steroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss. Results 101 patients underwent a first and 22 a second renal transplant. SRNS relapsed in 5/9 (55%) patients with disease recurrence in their first transplant and 2 of them lost the second graft. Conclusions Absence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. Retransplantation represents a second chance after graft loss for recurrence

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