Vascularite à IgA (purpura rhumatoïde) l’adulte

Abstract

IgA vasculitis is a systemic vasculitis affecting small vessels. IgA vasculitis 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 IgA vasculitis is unknown and its annual incidence is 1 in 1 million. The dominant clinical features include cutaneous purpura, arthritis and gastrointestinal symptoms. Sometimes 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 IgA vasculitis. Only symptomatic treatment is advised in case of self-limited disease. Treatment of severe IgA vasculitis, 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 of 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, as for IgA nephropathy. Some authors even suggest that IgA nephropathy and IgA vasculitis would be the same disease.