Long-term outcome of focal segmental glomerulosclerosis after pediatric renal transplantation

Abstract

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation can limit graft survival. Despite new immunosuppressive agents, the incidence of recurrence remains relatively high. To identify risk factors for recurrence and efficacy of treatment, we reviewed the outcome of 23 grafts in 16 children with FSGS who had undergone transplantation between 1985 and 2007 at La Paz Children's Hospital. Recurrence was 56.3% after the first transplantation. We did not find significant differences in age at diagnosis, age at transplantation, age at end-stage renal disease (ESRD), progression to ESRD, bilateral nephrectomy of native kidneys prior to transplantation, use of induction therapy or of different immunosuppressive regimens between patients with and without recurrence. Plasmapheresis (PP) was carried out in seven of nine patients who had suffered recurrence, achieving remission in six of them. One patient received high doses of cyclosporin (CsA) and plasmapheresis, attaining remission. Graft survival was lower (P = 0.043) in patients with FSGS than in those with other ESRD etiologies (first year 75% vs 91%; fifth year 44% vs 78%). Recurrence of FSGS limited graft survival (first year 66% vs 85%; third year 20% vs 68%) (P = 0.07). In our experience, PP can be effective in treating FSGS recurrence, although its effect on long-term graft survival seems more limited.

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.…”

Focal Segmental Glomerulosclerosis (FSGS) Recurrence in Kidney Allograft Recipients

“…Some centers have added cyclophosphamide and/or mycophenolate mofetil (MMF -2 g/day in two divided doses) to the PP protocol, but with variable results (40; 41). In report by Fuentes et al FSGS recurrence rate was 56.3% after the first and 80% in the second transplant in children (33). Plasmapheresis treatment was carried out in 7 of 9 patients, achieving remission in six of them.…”

“…Recurrence of FSGS limited graft survival (first year 66% vs 85%, third year 20% vs 68%). The report shows that PP can be effective in treating FSGS recurrence in children, although its effect on long-term graft survival seems to be more limited (33). Matalon et al in a group of 13 adult renal transplant recipients from 3 transplant centers who underwent PP for recurrent FSGS found 50% or greater decline in proteinuria in 7 of 13 patients, but 3 of them (23%) remained PPdependent (and required PP treatments every 2-4 weeks for up to 6 years to prevent progressive increase in proteinuria) (48).…”

“…FSGS patients who receive renal transplants have a 2-fold higher risk of losing graft at 10 years, compared to all patients transplanted for glomerulonephritis (37; 69). In report of Fuentes et al in pediatric patients with recurrent FSGS graft survival was lower than in those with other etiologies of CKD (first year 75% vs 91%; 5 th year 44% vs 78%) (33). In Schachter's material recurrence rate was 23% of patients with biopsy proven primary FSGS (55).…”

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]. Treatment with plasma exchange reduces proteinuria and prolongs graft survival in pediatric case series and is most successful in preserving renal function in patients who are treated early after recurrence is diagnosed [42,[45][46][47][48].…”