http://www.sciencedirect.com/science/article/pii/S1568997214001190
Highlights
• Autoimmune disease is a common manifestation of combined variable immunodeficiency.
• The co-existence of hypo- and hyper-immune states in the same individual at the same point in time is not implausible given the complexity of the immune system.
• Both T and B cells abnormalities may contribute to the development of autoimmunity in CVID patients.
• Increased autoreactive B cells and reduced T regulatory cells may be involved in the pathogenesis of autoimmunity in CVID.
The most common autoimmune disease found in CVID patients is autoimmune cytopenia, but rheumatoid arthritis, lupus, and now primary biliary cirrhosis have also been reported. The coexistence of immunodeficiency and autoimmunity appears paradoxical, since one represents a hypoimmune state and the other a hyperimmune state. However, this paradox may not actually be all that implausible due to the complex nature of immune cells, signaling pathways and their interactions. The cellular alterations in combined variable immunodeficiency include a range of T and B cell abnormalities. Selective immune derangements found in CVID include a downregulation of regulatory T cells (Treg cells), accelerated T cell apoptosis, abnormal cytokine production secondary to cytokine gene polymorphisms and increased autoreactive B cell production. The impact of these abnormalities on T and B cell interaction may not only explain the immunodeficiency but also the development of autoimmunity ...