Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria

To evaluate the therapeutic role of cyclosporin A (CyA) for the treatment of Henoch-Schönlein nephritis (HSN), 29 patients (18 boys, 11 girls) with nephrotic-range proteinuria were analyzed retrospectively. Mean age was 8.6 years (range 2.0-15.5 years) at diagnosis of Henoch-Schönlein purpura (HSP). All patients had developed the nephrotic-range proteinuria at a mean interval of 4.4 months (range 0-50.7 months) after the diagnosis of HSP. Mean duration of CyA treatment was 12.3 months (range 2.6-55.0 months). Mean follow-up times were 3.7 years (range 1.2-12.9 years) from the beginning of the CyA treatment. Steroids were tapered off and stopped gradually after initiation of CyA. All patients responded to the CyA treatment within a mean of 1.8 months (range 1 week to 3.5 months). Twenty-three patients achieved stable remission with mean follow-up duration of 3.2 years and 6 patients seemed to become CyA-dependent, since they developed proteinuria when the treatment was stopped. Renal function was preserved in all patients but one who developed end-stage renal disease after poor compliance with CyA. We concluded that CyA treatment for HSN showing nephrotic-range proteinuria is very effective and a safe method, although some patients become CyA-dependent.