TY - JOUR
T1 - Autoimmunity in common variable immunodeficiency
AU - Agarwal, Shradha
AU - Cunningham-Rundles, Charlotte
N1 - Funding Information:
This work was supported by National Institutes of Health grants AI-101093, AI-467320, and AI-48693 and National Institute of Allergy and Infectious Diseases contract 03-22 (to Dr. Cunningham-Rundles).
PY - 2009
Y1 - 2009
N2 - Common variable immunodeficiency (CVID) is the most common clinically significant primary immune defect. Although the hallmark of CVID is hypogammaglobulinemia, the intrinsic dysregulation of the immune system leads to defective T-cell activation and proliferation, as well as dendritic cell and cytokine defects. Although 70% to 80% of patients have had recurrent sinopulmonary infections, autoimmunity and inflammatory complications are also common. The most common autoimmune conditions are immune thrombocytopenic purpura and hemolytic anemia, but other autoimmune complications arise, including rheumatoid arthritis, pernicious anemia, primary biliary cirrhosis, thyroiditis, sicca syndrome, systemic lupus, and inflammatory bowel disease. Treatment of autoimmunity includes highdose immunoglobulins, corticosteroids, selected immu no suppressants, and other immune modulators. This review focuses on autoimmune conditions associated with CVID, potential mechanisms of immune dysregulation, and therapeutic strategies.
AB - Common variable immunodeficiency (CVID) is the most common clinically significant primary immune defect. Although the hallmark of CVID is hypogammaglobulinemia, the intrinsic dysregulation of the immune system leads to defective T-cell activation and proliferation, as well as dendritic cell and cytokine defects. Although 70% to 80% of patients have had recurrent sinopulmonary infections, autoimmunity and inflammatory complications are also common. The most common autoimmune conditions are immune thrombocytopenic purpura and hemolytic anemia, but other autoimmune complications arise, including rheumatoid arthritis, pernicious anemia, primary biliary cirrhosis, thyroiditis, sicca syndrome, systemic lupus, and inflammatory bowel disease. Treatment of autoimmunity includes highdose immunoglobulins, corticosteroids, selected immu no suppressants, and other immune modulators. This review focuses on autoimmune conditions associated with CVID, potential mechanisms of immune dysregulation, and therapeutic strategies.
UR - http://www.scopus.com/inward/record.url?scp=68949148856&partnerID=8YFLogxK
U2 - 10.1007/s11882-009-0051-0
DO - 10.1007/s11882-009-0051-0
M3 - Review article
C2 - 19671377
AN - SCOPUS:68949148856
SN - 1529-7322
VL - 9
SP - 347
EP - 352
JO - Current Allergy and Asthma Reports
JF - Current Allergy and Asthma Reports
IS - 5
ER -