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Clinical features of Henoch-Schönlein purpura. Italian Group of Renal Immunopathology.

Henoch-Schönlein purpura (HSP) is a vasculitis that often involves the kidneys with a IgA nephropathy indistinguishable from Berger's disease. It can affect patients of any age, even though an uneven distribution of prevalence and severity of renal disease is commonly observed as the renal involvement with transient hematuria without renal functional impairment is much more frequent in children than in adults. The Italian Group of Renal Immunopathology analyzed a cohort of HSP patients with the homogeneous criterium of a renal disease severe enough to indicate renal biopsy both in adults and in children. We report the clinical features of 219 HSP patients (136 adults and 83 children) enrolled from 43 Italian Centers of Nephrology. The diagnosis was based on the detection of IgA nephropathy in a clinical setting of palpable purpura and/or bowel angina. The peak age was in second decade (28% of the patients). In 37% of the cases a potential eliciting factor was identified, mostly infectious (41% in children vs 23% in adults, p < 0.05). A seasoned incidence was found in June (20% of the cases). Most patients had palpable purpura (96.3%), while bowel angina was reported in 54.1% and arthritis in 50.2% at onset. The full extrarenal syndrome was more frequent in children (59%) than in adults (47%, p < 0.05). The extrarenal signs were often not coincident with urinary manifestations. The clinical onset with nephrotic syndrome was more common in children than in adults (35% vs 24.3% respectively, p < 0.02) who more often presented with minimal or moderate proteinuria. The frequency of impaired renal function at onset was similar in both adults and children (24.2% and 31.2% respectively).