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.