Treatment of Henoch-Schonlein purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide

Background: Henoch-Schonlein Purpura (HSP) is a common childhood vasculitis with manifestations in numerous organ systems, including glomerulonephriti s. Patients with more severe HSP-associated glomerulonephritis may develop chronic renal failure. Currently, no widely accepted treatment protocols ex ist for patients with significant renal involvement. Methods: We retrospect ively reviewed the clinical courses of 12 children (mean age 9 years) with HSP glomerulonephritis treated with high-dose corticosteroids plus oral cyc lophosphamide, All patients had nephrotic-range proteinuria, and all had si gnificant histopathologic changes on biopsy, including crescentic nephritis in 10 patients. Treatment consisted of either intravenous pulse methylpred nisolone or oral prednisone followed by oral cyclophosphamide (2 mg/kg/day) for 12 weeks, along with either daily or alternate-day oral prednisone. Pr ednisone was tapered following completion of cyclophsophamide. Results: Ser um albumin rose significantly after treatment from 2.8 +/- (SD) 0.5 to 3.7 +/- 0.4 g/dl (p < 0.001), and there was a concurrent reduction in proteinur ia, as reflected by decreasing serial protein-to-creatinine ratios: from 6. 3 +/- 4.4 to 0.8 +/- 0.8 (p = 0.002). Renal function remained normal in all patients. Hypertension developed during treatment in 10 patients, all but 1 of whom were normotensive at last follow-up, 35 +/- 17 months following b iopsy. Conclusions: We conclude that treatment of children with HSP nephrit is with high-dose corticosteroids plus oral cyclophosphamide is safe and, a s in nephrotic syndrome, appears to significantly reduce proteinuria which is a known risk factor for the development of renal insufficiency in HSP. F urther studies with larger numbers of patients should be conducted to confi rm this finding. Copyright (C) 2001 S. KargerAG, Basel.