ABSTRACT
Twenty patients with severe Schönlein-Henoch nephritis were selected on a histologically basis of diffuse proliferative endo- and/or extracapillary glomerulonephritis during a period of 12 years in Champagne-Ardenne. There were 15 men and 5 women, mean age 44.1 years. An infectious history was found in 40%, an urinary tract cancer in 15%. In all cases there was purpura, in 80% joint pain and in 50% digestive symptoms. Clinical presentation at diagnosis included, in all cases, hematuria (gross in 50%) and proteinuria (of nephrotic range in 80%); there was hypertension in 60% and renal failure in 80%. Histology found, in all cases, mesangial IgA and often C3 deposits, with a diffuse endocapillary proliferation in 10%, extra-capillary proliferation in 30% and both endo-extracapillary in 60%; 45% of the patients had crescents in greater than 50% of glomeruli. The outcome, after steroid and immunosuppressive treatment, was end-stage renal failure in 25%, moderate renal failure in 20%, or normal renal function in 55% with a mean follow-up period of 4.6 years. These severe nephritis were associated with repetitive and often necrotic purpura, frequent joint pain and severe digestive symptoms. The analysis of initial renal presentation confirmed the bad prognosis of nephrotic syndrome, renal failure and especially hypertension, which were well correlated with the severity and diffusion of proliferative lesions. Despite a worse known prognosis, these nephritis responded to an aggressive and early treatment.
