Single-centre experience with cyclosporin in 106 children with idiopathic focal segmental glomerulosclerosis

Background. Although remission is achieved in most children with nephrotic syndrome by treatment with corticosteroids, a significant proportion of patients experience relapses. Continuous or repeated use of corticosteroids inevitably induces features of steroid side-effects. Cyclosporin (CsA) has been used in the treatment of idiopathic steroid-dependent and -resistant nephrotic syndrome. However, relapse often occurs shortly after the CsA treatment is terminated. Furthermore, long-term clinical outcome of patients treated with CsA is unclear. Methods. We retrospectively reviewed the data of 106 nephrotic children having primary focal segmental glomerulosclerosis (FSGS) who received CsA between 1993 and 2002. Indications of CsA therapy were steroid resistance (n = 45) and steroid dependence with steroid toxicity (n = 61). Fifty-four patients received cyclophosphamide prior to CsA therapy. CsA starting dose was 6 mg/kg/day to be readjusted to maintain a whole blood trough level of 80–150 ng/ml. The drug was received for 6–48 months (mean: 22.1±11 months). The observation period was 5.8±3 and 6.1±1.9 years before and after CsA treatment, respectively. Results. Complete remission [proteinuria