Update on immunoglobulin A vasculitis

Abstract

Immunoglobulin A vasculitis (IgAV) is a systemic vasculitis of the small vessels with formation of IgA immune complexes and a broad spectrum of clinical constellations. Typical manifestations include purpura, arthralgia or arthritis, enteritis and glomerulonephritis. The IgAV is the most common vasculitis in childhood with a mostly uncomplicated and self-limiting course. In adults IgAV is much less frequent but the course can be more complicated, especially with renal or gastrointestinal manifestations. Various triggers of IgAV including infections have been described, whereby impaired glycosylation of IgA1 with subsequent exposure of binding sites for autoantibodies is a pathophysiological precondition for the vasculitis. Therapeutic strategies with immunosuppressants are so far supported by low evidence, take the severity of the organ manifestations into account and are oriented towards the recommendations for the treatment of other vasculitides of small vessels. Benign courses are treated symptomatically. The long-term prognosis of IgAV is determined by the renal manifestation.