Long-term Cyclosporine A (Sandimmun) therapy for steroid resistant nephrotic syndrome in children.

The aim of the paper was to evaluate Cyclosporine A (CsA) treatment in children with steroid resistant nephrotic syndrome (diagnosed according to criteria established by ISKDC), in whom numerous attempts at other types of therapy had failed. CsA was used in 23 children aged 2-16 years. Renal biopsies revealed minimal changes (MCD) in 7 children, focal segmental glomerulosclerosis (FGS) in 11, mesangial glomerulonephritis (MES) in 4, and membrano-proliferative glomerulonephritis (MPGN) in 1. CsA was administered for a period of 6-41 months at a daily dose of 6 mg/kg, gradually decreased to 2.5 mg/kg. Blood CsA level was 40-200 ng/ml. In the course of the treatment, proteinuria disappeared or diminished and clinical improvement was seen in 21 patients. 13 children had relapses of nephrotic syndrome, showing very low blood CsA levels. Complete remissions were achieved in 16 children, partial remissions in 5, no remission in 2. Throughout the treatment and after its cessation 22 patients manifested no deterioration of renal function. In 1 child, repeated renal biopsies revealed some lesions that could suggest a nephrotoxic effect of CsA. Secondary steroid sensitivity observed in 3 children should be emphasized. Concluding, CsA is an effective and well tolerated agent in children with steroid resistant nephrotic syndrome, and the improvement noted in patients with FGS may reduce the risk of early terminal failure. The treatment requires monitoring blood CsA levels.