Methylprednisolone or cyclosporine a in the treatment of Henoch-Schönlein nephritis: a nationwide study

Background Optimal treatment of Henoch-Schönlein purpura nephritis (HSN) remains unclear. We evaluated outcome of pediatric HSN patients treated initially with either methylprednisolone (MP) or cyclosporine A (CyA) in Finland between 1996 and 2011. Methods Outcome of 62 HSN patients was evaluated by screening urine and blood samples ( n = 51) or by collecting clinical parameters from medical charts until last follow-up visit ( n = 11). Sixty (97%) patients had nephrotic-range proteinuria and/or ISKDC grade ≥ III before initial treatment. Patients were initially treated with either MP pulses ( n = 42) followed by oral prednisone or with CyA ( n = 20). Fifty-nine (95%) patients received angiotensin-converting enzyme inhibitors and/or angiotensin receptor blockers. Results Mean follow-up time was 10.8 years (range 3.2–21.2 years). One patient developed end-stage renal disease and another had decreased renal function (eGFR 0.5 g/day at end of follow-up. Sixteen (38%) MP-treated and two (10%) CyA-treated patients needed additional immunosuppressive treatment (RR 3.81, 95% CI 1.16–14.3, p = 0.035). Late initiation of treatment was associated with an increased risk for persistent proteinuria. Conclusions Long-term outcome was relatively good in both treatment groups. However, since urinary abnormalities may persist or develop, long-term follow-up of HSN patients is mandatory. Early initiation of treatment had a favorable effect on proteinuria.