Long-term effects of cyclosporine A in Alport's syndrome

Background. In 1991, our initial results of cyclosporine A (CsA) administra tion in eight patients with Alport's syndrome were published. A significant decrease in or disappearance of proteinuria and apparently good tolerance to CsA were observed in all patients. Methods. CsA administration has been maintained in these eight patients wit h the aim of obtaining further information about the clinical course of the disease. The ages of these eight patients currently range from 15 to 27 ye ars, and the mean duration of treatment is from 7 to 10 years (x = 8.4 year s). Results. Renal function has remained stable, with no evaluable changes in s erum creatinine levels compared with pre-CsA treatment values. Proteinuria in all patients has either remained negative or are values far lower than p retreatment levels. A second renal biopsy was performed in all patients aft er five pears of CsA administration. No aggravation of the lesion present a t the first biopsy or lesions typical of cyclosporine intoxication was obse rved. Conclusions. After a mean duration of 8.4 years and with no deterioration i n renal function, we found possible beneficial effects of the continued tre atment of CsA in patients with Alport's syndrome who present evidence of pr ogression to renal insufficiency.