Selective IgA Deficiency in Autoimmune Diseases

Review Article

Ning Wang, Nan Shen, Timothy J Vyse, Vidya Anand, Iva Gunnarson, Gunnar Sturfelt, Solbritt Rantapää-Dahlqvist, Kerstin Elvin, Lennart Truedsson, Bengt A Andersson, Charlotte Dahle, Eva Örtqvist, Peter K Gregersen, Timothy W Behrens, and Lennart Hammarström

Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. It has previously been suggested to be associated with a variety of concomitant autoimmune diseases. In this review, we present data on the prevalence of IgAD in patients with Graves disease (GD), systemic lupus erythematosus (SLE), type 1 diabetes (T1D), celiac disease (CD), myasthenia gravis (MG) and rheumatoid arthritis (RA) on the basis of both our own recent large-scale screening results and literature data. Genetic factors are important for the development of both IgAD and various autoimmune disorders, including GD, SLE, T1D, CD, MG and RA, and a strong association with the major histocompatibility complex (MHC) region has been reported. In addition, non-MHC genes, such as interferon-induced helicase 1 (IFIH1) and c-type lectin domain family 16, member A (CLEC16A), are also associated with the development of IgAD and some of the above diseases. This indicates a possible common genetic background. In this review, we present suggestive evidence for a shared genetic predisposition between these disorders.

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Posted by Leah Caracappa on Dec 12, 2011 12:00 AM CST