Abstract
Immunoglobulin A (IgA) nephropathy (IgAN) is a mesangial proliferative glomerulonephritis characterized by diffuse mesangial deposition of IgA. IgA vasculitis (IgAV), previously termed Henoch-Schönlein purpura, is a small-vessel vasculitis affecting the skin, joints, gut, and kidneys that predominantly affects children. The pathogenesis of both includes a genetic predisposition and the occurrence of undergalactosylated IgA, which deposits in the glomerular mesangium. Most patients with IgAN are oligosymptomatic with microhematuria, usually nonnephrotic proteinuria, hypertension, and/or reduced kidney function. End-stage kidney disease may develop. Clinical features and histologic findings based on the Oxford classification may be used to predict the outcome. Therapy chiefly includes supportive measures. The value of corticosteroids and other immunosuppressive agents is less well established. IgAN may recur after kidney transplantation in patients with IgAN or IgAV.
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