Effectiveness and safety of cyclosporin A therapy in steroid dependent nephrotic syndrome in childhood

Objective: To analyse the effectiveness and safety of cyclosporin A (CsA) in the treatment of steroid dependent nephrotic syndrome (SDNS) in childhood.

Design: Prospective descriptive study

Setting: Nephrotic Syndrome Clinic, Teaching Hospital Peradeniya, Sri Lanka

Method: Children with SDNS who had normal renal function, relapsed while receiving over 1mg/kg prednisolone on alternate days and who had received at least one course of cyclophosphamide were recruited to the study over a period of 5 years. They were treated with CsA 3-5 mg/kg/day orally with a tapering dose of alternate day prednisolone. Statistical analysis was performed using paired t-test.

Results: Over 5 years, 48 children who satisfied the inclusion criteria were recruited. CsA therapy was discontinued in two patients who had evidence of nephrotoxicity within 6 months of therapy. Forty six patients completed the study. Thirty one were male. The maintenance dose of prednisolone was tapered at least by 50% at 6 months of therapy in 38 (83%) patients. At the completion of 6 months of therapy, the mean serum cholesterol decreased from 396.96 mg/dl to 269.5 mg/dl (p<0.01) and the mean serum albumin increased from 21.1 mg/dl to 33.5 mg/dl (p<0.01). Hypertension was seen in 26 (57%) patients and hirsutism in 37 (80%). All patients had normal liver enzymes and opportunistic infections were not encountered.

Conclusions: CsA treatment in combination with low-dose prednisolone is highly effective in maintaining complete remission in difficult SDNS in childhood. Reversible nephrotoxicity was seen in 4% of patients, hypertension in 57% and hirsutism in 80%.

(Key words: Nephrotic syndrome; steroid dependence; cyclosporin A; relapse)

DOI: http://dx.doi.org/10.4038/sljch.v41i4.4978