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Background Corticosteroids have been shown not to prevent the development of Henoch-Schonlein nephritis. However, long-term follow-up data are scarce. Methods The long-term outcome of patients in a randomized placebo-controlled prednisone study was evaluated 8 years later with a health questionnaire completed by 160/171 (94%) patients and by urine and blood pressure screening (138/171, 81%). Results Twelve patients had hematuria and/or proteinuria and seven had hypertension. The patients with nephritis at onset of Henoch-Schonlein purpura (HSP) had an increased risk of hypertension and/or urine abnormalities (odds ratio 3.6, p = 0.022, 95% confidence interval 1.3-10.0). There were no differences between the prednisone and placebo groups. Recurrences of purpura were reported by 15 patients, with some recurrences continuing for 10 years. All five reported pregnancies were complicated by proteinuria. Four patients presented with hematuria and/or proteinuria at the control visit, and four had hypertension. Of these, two had a decreased estimated glomerular filtration rate. Conclusions HSP has a good long-term prognosis in unselected patients, although skin relapses with/without late-onset nephritis may occur, even a decade after the initial disease. Urine and blood pressure abnormalities 8 years after HSP are associated with nephritis at its onset. Early prednisone treatment does not affect the outcome and should not be routinely used. Keywords Vasculitis * Glomerulonephritis * Hypertension * Recurrences * Pregnancy * Familial cases * Corticosteroids, Introduction Henoch-Schonlein purpura (HSP) is the most common form of pediatric vasculitis and accounts for half of all vasculitides in children [1]. HSP vasculitis of small vessels is mediated by [...]

Knowledge about how to treat severe Henoch-Schönlein nephritis (HSN) is scarce. The aim of our study is to compare cyclosporine A (CyA) and methylprednisolone pulses (MP) in the treatment of severe HSN. Out of 24 pediatric HSN patients with nephrotic-range proteinuria or crescentic HSN in kidney biopsy, seven were randomized to receive CyA for 12 months at an initial dose of 5 mg/kg and eight to receive 3 MP pulses of 30 mg/kg followed by prednisone for 4 months. The other nine patients received identical treatment without randomization. Kidney biopsies were performed at inclusion and after 2 years. The primary outcomes were the duration of proteinuria and hematuria, estimated glomerular filtration rate, and renal biopsy histology. All the 11 CyA-treated patients achieved resolution of nephrotic-range proteinuria within 3 months, while the MP-group response was slower, and in 6/13 was not achieved with the initial treatment. Additional immunosuppressive treatment was needed in none of the CyA-treated patients but in six patients treated with MP (difference in proportion 46%, p = 0.008). The 2-year control biopsies were similarly improved in both groups. After mean 6.1 years (2.2-10.4 years), 16 patients (eight CyA, eight MP) had no renal symptoms and six (three CyA, three MP) had persistent nephropathy but normal renal function. One MP-treated patient had reduced renal function and another had developed ESRD and received a renal transplant. CyA gave a 100% resolution of nephrotic-range proteinuria and a 100% renal survival rate without additional therapy after a mean follow-up of 6 years. Treatment of HSN with CyA is efficacious, safe and not inferior to MP., Author(s): Outi Jauhola [sup.1] , Jaana Ronkainen [sup.2] , Helena Autio-Harmainen [sup.3] , Olli Koskimies [sup.4] , Marja Ala-Houhala [sup.5] , Pekka Arikoski [sup.6] , Tuula Hölttä [sup.4] , Timo [...]

Unfortunately Table 1 was printed incorrectly. The correct version can be seen below., Author(s): Outi Jauhola [sup.1] , Jaana Ronkainen [sup.2] , Helena Autio-Harmainen [sup.3] , Olli Koskimies [sup.4] , Marja Ala-Houhala [sup.5] , Pekka Arikoski [sup.6] , Tuula Hölttä [sup.4] , Timo [...]