High-dose oral cyclosporin therapy for recurrent focal segmental glomerulosclerosis in children.

BACKGROUND

Focal segmental glomerular sclerosis (FSGS) has a high propensity for recurrence after renal transplantation, with a 50% risk for graft failure from recurrent disease.

METHODS

We report on the efficacy of high-dose oral cyclosporin A (CsA) in the treatment of recurrent FSGS in children. Between August 1991 and January 2003, a total of 24 patients with FSGS underwent transplantation at 1 institution. Sixteen patients (67%) had recurrent disease. In these 16 patients, CsA dose was increased gradually until remission was achieved or there was evidence of renal toxicity. Seven patients also underwent plasma exchange.

RESULTS

Thirteen patients (81%) achieved remission. Remission was complete in 11 patients and partial in 2 patients. The CsA dose necessary for inducing remission ranged from 6 to 25 mg/kg/d. Four of these patients also underwent plasma exchange. After remission, CsA dose was reduced gradually toward the standard posttransplantation regimen. Eleven of 13 responders have a functioning graft after a follow-up ranging from 10 months to 12 years. One graft was lost because of recurrent FSGS, and another graft because of recurrence and cellular rejection; noncompliance was a factor in both losses. The 3 patients with disease that did not respond to high-dose CsA therapy lost their grafts because of recurrent FSGS. A common factor in these 3 patients was the inability to increase the CsA dose because of early evidence of nephrotoxicity, evidenced by an increase in serum creatinine level.

CONCLUSION

High-dose oral CsA therapy is effective in producing long-lasting remission of recurrent nephrotic syndrome in children with FSGS.