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Current Opinion in Allergy & Clinical Immunology:
doi: 10.1097/ACI.0b013e3283594219
PRIMARY IMMUNE DEFICIENCY DISEASE: Edited by Amos Etzioni and Ramsey L. Fuleihan

IgA deficiency: what is new?

Wang, Ning; Hammarström, Lennart

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Abstract

Purpose of review: To summarize recent publications on clinical and genetic aspects of IgA deficiency (IgAD).

Recent findings: Both major histocompatibility complex (MHC) and non-MHC genes contribute to susceptibility to the disease. The former genes appear to be located in different parts of the MHC region depending on the HLA haplotype. The latter show a marked overlap with genes associated with a variety of autoimmune disorders including Graves’ disease, systemic lupus erythematosus, type 1 diabetes and celiac disease, suggesting common pathophysiological mechanisms. Various cytokines, recently shown to include interleukin 21, can induce IgA synthesis in vitro in cells from patients with IgAD, suggesting a regulatory basis of the disease.

Summary: IgAD is the most common primary immunodeficiency in the Western world with a prevalence of approximately 1 : 600 in the general population. It appears to be a polygenic disorder and several of the genes involved have recently been identified. The involvement of genes associated with autoimmunity may suggest that IgAD in itself is an autoimmune disease.

© 2012 Lippincott Williams & Wilkins, Inc.

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