[Adult Henoch-Schönlein purpura].
Henoch-Schönlein purpura (HSP) is a systemic IgA vasculitis affecting small vessels. HSP usually affect children whereas it is rare in adults (150 to 200 for 1) in which the disease is often more serious with more frequent and severe nephritis. Prevalence of adult PR is unknown and its annual incidence is 1 in 1 million. The dominant clinical features include cutaneous purpura, arthritis and gastrointestinal symptoms. Some times nephritis can add, typically as glomerulonephritis with IgA mesangial deposits. Pulmonary, cardiac, genital and neurological symptoms have also been observed. Although the cause is unknown, it is clear that IgA plays a pivotal role in the immunopathogenesis of HSP. Only symptomatic treatment is advised in case of self limited disease. Treatment of severe HSP, nephritis or gastrointestinal manifestations, is not established but some studies, which need to be confirmed, reported the benefit of corticosteroids combined with immunosuppressive drugs. Short term outcome depends on the severity of the gastro-intestinal manifestations. The long term prognosis is heavily dependent on the presence and severity of nephritis. Studies with prolonged follow-up show up to one third of adult patients reaching end stage renal failure.
Pillebout
Evangéline
E
Service de néphrologie et de transplantation, Hôpital Saint-Louis, F-75010 Paris, France. evangeline.pillebout@sls.ap-hop-paris.fr
D003937:Diagnosis, Differential; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D011379:Prognosis