Common variable immune deficiency and autoimmunity.
Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by various degrees of hypogammaglobulinemia. Similar to many immunodeficiency disorders, autoimmunity is common with an association with autoimmune cytopenias, a sarcoidosis-like disorder and inflammatory bowel disease. Recent efforts have characterized selective immunological defects and genetic associations in CVID and demonstrate an increased tendency towards loss of tolerance. The mainstay of treatment of autoimmune disease in such patients is often high dose IVIG and corticosteroids, although other therapies, including TNF-alpha antagonists, have been reported. While the etiology of increased autoimmunity in CVID remains elusive, certain genetic predispositions in combination with repeated antigen exposure and overall immune dysregulation inherent in CVID likely play a significant role.
Brandt
Daniel
D
Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 E. Health Sciences Drive, Suite 6510, Davis, CA 95616, USA.
Gershwin
M Eric
ME
D000818:Animals; D015551:Autoimmunity / Q000235:genetics / Q000502:physiology*; D017074:Common Variable Immunodeficiency / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy; D006801:Humans