Which autoimmune conditions are associated with common variable immunodeficiency (CVID)?

Updated: Oct 16, 2018
  • Author: C Lucy Park, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

In contrast to X-linked agammaglobulinemia (XLA), common variable immunodeficiency is associated with a high frequency of autoimmune manifestation. Resnick reported autoimmunity in about 28.6% with one or more autoimmune manifestation.

The most common autoimmune conditions in patients with common variable immunodeficiency are cytopenia, idiopathic thrombocytopenic purpura (ITP) in particular, and hemolytic anemia or, more rarely, autoimmune neutropenia. Other solid organ–specific autoimmune diseases (eg, pernicious anemia, thyroid diseases, vitiligo) have prevalence rates of more than 5% in patients with common variable immunodeficiency, which is higher than in the general population. Other conditions include insulin-dependent diabetes, psoriasis, systemic lupus erythematosus, rheumatoid arthritis, juvenile rheumatoid arthritis, and uveitis. Approximately 20% of patients have a severe gastroenteropathy with severe malabsorption, nodular lymphoid hyperplasia, and chronic inflammatory bowel disease, such as ulcerative colitis and Crohn disease.

Although regular Ig replacement therapy reduces susceptibility to Giardia species and Campylobacter enteritis, it does not prevent autoimmune mucosal inflammation. Ig replacement therapy does not affect the clinical course of inflammatory bowel disease.

Patients who have common variable immunodeficiency and autoimmune conditions appear to have very low numbers of isotype-switched memory B cells in peripheral blood and are more likely to have a mutation in the gene that encodes TACI (TNFRST13B).


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