Effect of cyclosporin A on proteinuria in patients with Alport's syndrome.

Eight patients with Alport's syndrome and massive proteinuria (129 +/- 60.57 mg/m2 per hour) were treated with cyclosporin A (CyA) for 8 months. The average dose of CyA administered to all patients was 4.21 +/- 0.26 mg/kg per day and blood CyA levels of 63.4 +/- 4.1 ng/ml were attained. In five patients, proteinuria abated during the 3rd week of treatment. In the remaining three, all of whom had low creatinine clearance (82.0, 46.0 and 43.2 ml/min per 1.73 m2 respectively), proteinuria persisted but at levels lower than before treatment: 32.5 +/- 15.9 mg/m2 per hour versus 183.3 +/- 29.7 mg/m2 per hour. No permanent decrease in creatinine clearance was observed in any of these patients throughout treatment. In those patients in whom proteinuria abated, it reappeared 2 weeks after discontinuation of CyA treatment. We observed no significant increases in angiotensin II plasma levels in our patients during CyA administration. Although we have shown that CyA will reduce massive proteinuria in patients with Alport's syndrome, we cannot yet recommend its use as a therapeutic measure.