Adult IgA Vasculitis (Henoch-Schönlein Purpura)

Abstract

IgA vasculitis (IgAV) is a systemic IgA vasculitis affecting small vessels. IgAV usually affects children; it is rare in adults (150–200 for 1). The disease is often more serious in adults with more frequent and severe nephritis. Prevalence of adult IgAV is unknown and its annual incidence is 1 in 1 million. The dominant clinical features include cutaneous purpura, arthritis, and gastrointestinal symptoms. Sometimes nephritis occurs, 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 IgAV. Only symptomatic treatment is advised in case of self-limited disease. Treatment of severe IgAV, 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 gastrointestinal 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.