Abstract
Summary: IgA nephropathy is a clinically and histologically defined syndrome of unknown aetiology, which may have various causes in different parts of the world. Immunologically it is characterized by deposition of IgA1, probably polymeric IgA, in the mesangium and is frequently associated with IgG, C3 and components of the alternative pathway of the complement cascade. the disease can go into complete remission in children, but in adults it usually has a progressive course, characterized by the appearance of proteinuria and hypertension and loss of glomerular filtration rate (GFR). Histologically the development of glomerulosclerosis and tubulo‐interstitial changes correlate with a clinical progressive course. the mucosal immune system is characterized by high plasma IgAl antibody responses after parenteral immunization with viral or bacterial vaccines. However, following nasal challenge with a bacterial neoantigen, IgA nephropathy patients appear to have a defective mucosal immune response in their nasal washes, in their bone marrow and in their plasma IgA1 antibody levels.
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