The use of cyclosporin A in adult nephrotic syndrome: nine cases and literature review

Nine adult patients with resistant nephrotic syndrome were treated with cyclosporin A (CyA). All had failed to respond to high dose corticosteroids with or without cyclophosphamide. Three patients had minimal change disease, 3 had focal sclerosing glomerulosclerosis (FSGS), 2 had mesangio-capillary GN, and one had membranous nephropathy. The mean age of the patients was 26.4 years (range 16 to 39 years). CyA was given orally twice daily at a mean dose of 6.7 mg/kg/24 hours (range 6–10 mg/kg/24 hours). Four patients achieved full remission, two patients went into partial remission, and three failed to respond. Two patients developed clinical nephrotoxicity, which reversed on dose reduction or cessation of CyA. All 3 patients with minimal change disease who responded subsequently relapsed after stopping CyA, but remitted rapidly on reintroduction of the drug. We suggest that the mode of action of CyA in nephrotic syndrome may be related to intra-renal vasoconstriction in addition to its direct immunosuppressive effect. In this limited series, we found that CyA can be an effective therapy for otherwise refractory nephrotic syndrome, although relapse on withdrawal of CyA may well be a significant clinical problem.