How is pathogenesis of IgA nephropathy?

➢ An abnormally glycosylated IgA1 immunoglobulin is thought to play a central role in the pathogenesis. ➢ This abnormal IgA may elicit an autoimmune response, and autoantibodies may form large immune complexes with circulating IgA. ➢ These complexes deposit in the glomerular mesangium; ➢ This unusual location may be related to physicochemical features of the IgA and may be facilitated by an IgA1 receptor (CD71) on mesangial cells. ➢ IgA nephropathy occurs with increased frequency in individuals with celiac disease, in whom intestinal mucosal defects are seen, and in liver disease, in which there is defective hepatobiliary clearance of IgA complexes (secondary IgA nephropathy)