Recurrent focal glomerulosclerosis in pediatric renal allografts: the Miami experience

Abstract

Recurrence of focal glomerulosclerosis (FSGS) following renal transplantation is a common cause of allograft loss and clinical morbidity. Recent attempts to control proteinuria and morbidity with plasmapheresis (PP) have met with limited success. Our experience with the use of mycophenolate mofetil (MMF) and angiotensin blockade (AB) in the management of refractory FSGS pre transplant suggested its potential benefit in post-transplant recurrence. This report presents our 25-year experience in pediatric renal transplantation of patients with FSGS divided into two treatment eras: Era 1-prior to use of daclizumab (anti-IL-2R) and Era 2-after daclizumab. A total of 179 pediatric patients were transplanted during the 25-year period. FSGS was confirmed in 27 (15%); 16 of 28 allografts (57%) had recurrence of FSGS during the post-transplant period. In Era 1, only 6 of 16 (38%) recurred in the allograft, while 10 of 12 (83%) recurred during Era 2. The odds ratio of recurrence of FSGS in the allograft after induction with anti-IL-2R was 8.3 (95% confidence interval=1.3-52, P =0.02). Only 2 patients in Era 1 received PP, while 10 in Era 2 were entered into an intensive PP protocol followed by maintenance with AB consisting of angiotensin receptor blockers alone, or in combination with angiotensin-converting enzyme inhibitor. Although proteinuria decreased an average of 80+/-16% with PP, the response was variable and severe morbid edema persisted in poor responders. Maximum benefit occurred with the addition of AB and MMF. After a follow-up of 27+/-15 months, proteinuria has shown a sustained decrease of 94+/-8% below baseline. In conclusion, our experience suggests that, with recurrent FSGS, a limited course of PP followed by maintenance therapy with AB and MMF improves symptoms and may preserve allograft function.

Recurrent focal segmental glomerulosclerosis after kidney transplantation

“…Plasmapheresis Plasma-based treatments, primarily plasmapheresis, have been used as a first-line treatment for FSGS and for recurrent FSGS after transplantation for nearly 20 years [5,[46][47][48][49][50][51][52][53][54][55][56][57][58][59]. The results of select studies performed since the year 2000 with larger numbers of patients are summarized in Table 2.…”

Management of proteinuria in the transplanted patient

“…recurrence, rejection, hypertension). The precise recommendations for the treatment of these causes of proteinuria are beyond the scope of this review article and are covered comprehensively elsewhere [28,56,57,59].…”

“…Some studies showed an impaired graft survival in FSGS patients [29,55], whereas others did not find any differences in graft survival between recurrent FSGS patients and non-FSGS children [56,57]. Unpublished data from a retrospective study conducted in our transplantation center show that children (without recurrent FSGS) with pathological proteinuria at 1-year post-transplant have significantly worse long-term graft survival than those with normal proteinuria at 1-year post transplant.…”

Therapeutic plasma exchange for the treatment of pediatric renal diseases in 2013

“…FSGS accounts for 9 % of pediatric patients with ESRD receiving a transplant in North America, and children with ESRD secondary to FSGS have a high rate (30-86 %) of recurrence of proteinuria after renal transplant [39][40][41][42]. Graft failure is more common among patients with recurrence, and the duration of graft survival is lower among patients with FSGS than among those with other causes of glomerular disease [43,44].…”