Immunogenetic findings in glomerulonephritis

In the 19th century, several authors recognized that some renal diseases tend to run in families. Alport pointed to the constellation of nephritic urinary sediment, hearing loss and progression into renal failure. Today this is recognized to result from abnormal basement membrane (BM) collagen synthesis. Recently it has been recognized, however, that other forms of glomerulonephritis may also run in families. In about 10% of patients with glomerulonephritis one or more sibling also suffers from glomerulonephritis. Genetically determined derangements of immune regulation may play a role in the genesis of primary chronic glomerulonephritis. Potentially associated immunogenetic abnormalities include inherited defects of the complement system, increased prevalence of certain HLA-types, altered frequencies of polymorphisms in immunoglobulins and TCR genes and others. This overview summarizes immunogenetic studies performed in patients with glomerulonephritis with special emphasis on patients with mesangial IgA GN.