Deficiency

In Caucasians, IgA deficiency is the most common form of selective Ig isotype deficiency. Estimates vary between 1:300 and 1:800 in a normal population. Among the Japanese, IgA deficiency is reported to be much less frequent. Individuals with IgA deficiency have normal a chain structural genes, but may express a higher frequency of particular major histocompatibility complex (MHC) haplotypes. This suggests that IgA deficiency is associated with defects in immune regulation (e.g. secondary to cytokine deficiency) and evidence has been presented which indicates that IgA deficiency may be linked to the presence or absence of particular HLA haplotypes. In addition to generalized deficiency of IgA, selective deficiency in one or the other IgA isotype or in systemic versus mucosal IgA have been reported.

There appears to be general consensus that individuals with IgA deficiency are at risk for mucosal infection, although many such individuals are healthy. Individuals who develop a compensatory increase in IgM antibody synthesis appear to be indistinguishable from normal individuals with respect to frequency of mucosal infection. Infants who are transiently deficient in IgA in the first year of life are prone to develop atopic disease. An increased frequency of autoimmune diseases has also been reported in IgA-deficient individuals, which may reflect the importance of a putative defect in immune regulation, although increased exposure of the host to foreign antigens as a result of the loss of 'immune exclusion' may play a role. In contrast to humans, IgA deficiency has not been reported in many species. A group of IgA-deficient dogs has been reported.

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