LONG-TERM PROGNOSIS OF HENOCH-SCHÖNLEIN NEPHRITIS IN CHILDREN

Abstract

INTRODUCTION: The long-term prognosis in Henoch-Schönlein purpura is determined principally by the development of progressive glomerulonephritis (>10% progress to end-stage renal failure). OBJECTIVE: In this study we aimed to investigate the long-term prognosis of Henoch-Schönlein nephritis (HSN) in childhood. METHODS: Between 1991 and 2003, 156 patients with HSN were investigated retrospectively. RESULTS: There were 86 boys and 70 girls with a mean age of 9.6 years. They were graded according to the degree of renal involvement: grade 1, isolated microscopic hematuria (n = 31); grade 2, hematuria and mild proteinuria (n = 60); grade 3, acute nephritic syndrome (n = 4); grade 4, nephrotic syndrome ± hematuria (n = 18); grade 5, acute nephritic and nephrotic syndrome (n = 43). Renal biopsy was performed on 43 patients with grade 4 or 5 disease. Twenty patients had extensive crescent formation (>50%) as shown by the renal biopsy and were given triple therapy (intravenous pulse methylprednisolone [30 mg/kg per day for 3 days] followed by oral prednisolone [OP], oral cyclophosphamide [2 mg/kg per day for 2 to 3 months], and dipyridamole). The other 23 patients with <50% crescent formation were given methylprednisolone followed by OP and dipyridamole. The patients with grade 3 or 4 disease were given OP and dipyridamole. Those with grade 1 or 2 disease were not given any immunosuppressive agent. During the follow-up period (mean: 30 ± 3.5 months; range: 12–96 months), 23 patients with grade 1, 38 patients with grade 2, 2 patients with grade 3, 8 patients with grade 4, and 21 patients with grade 5 disease showed complete remission (59%). Of the 5 patients with extensive fibrosis shown by renal biopsy, 2 (1%) had persistent nephropathy and 3 (2%) developed end-stage renal failure. The remaining 59 patients showed near-complete recovery with minimal urinary abnormalities (38%). CONCLUSIONS: Although initial presentation of renal involvement determines the prognosis for those with HSN, intensive treatment with triple therapy seems to be effective for severe renal disease, especially if started before the development of fibrotic changes in crescents and tubulointerstitial tissue.