Immunoglobulin A (IgA) nephropathy was first described by the pathologist Jean Berger (1,2) and thus is sometimes called Berger's disease. Immunoglobulin A nephropathy is defined by the presence of IgA-dominant or co-dominant mesangial immunoglobulin deposits (Fig. 6.l) (3). Lupus glomerulonephritis, which may have IgA dominant or co-dominant deposits, is excluded from this diagnostic category. Immunoglobulin A nephropathy occurs as a primary (idiopathic) disease, as a component of Henoch-Schönlein purpura small-vessel vasculitis, secondary to liver disease (especially alcoholic cirrhosis), and associated with a variety of inflammatory diseases including ankylosing spondylitis, psoriasis, Reiter's disease, uveitis, enteritis (e.g., Yersinia enterocolitica infection), inflammatory bowel disease, celiac disease, dermatitis herpetiformis, and HIV infection (4-6).
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