IgA Vasculitis (Henoch-Schönlein Purpura)

IgA vasculitis (IgAV, Henoch-Schönlein purpura) is an IgA-mediated disease affecting small blood vessels, characterized by purpuric skin lesions unrelated to an underlying coagulopathy, abdominal pain and bleeding, arthritis, and renal involvement. Histologically, there is infiltration of the small blood vessels with polymorphonuclear leukocytes and the presence of leukocytoclasia and IgA-dominant immune deposits. It is the most common vasculitis in children and is infrequent in adults. It may follow an intercurrent illness, usually an upper respiratory tract infection. Skin, joints, GI, and kidneys are the organs most commonly involved. It is benign and self-limited in the vast majority of children and most adults, but a small percentage, especially adults, progress to renal insufficiency. For this reason, close follow-up with urinalysis is advisable in those who present with renal involvement during the first 6 months after the onset of the disease. Pharmacological treatments are controversial; no optimal management has been established for patients with renal disease or severe GI complications and the benefits of prophylactic glucocorticoid therapy to prevent renal disease are not clear. For a better understanding of management of IgAV, as well as precipitating factors and pathology, long-term studies are required.