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Autoimmunity in common variable immunodeficiency

  • Shradha Agarwal
  • Charlotte Cunningham-RundlesEmail author
Article

Abstract

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.

Keywords

Systemic Lupus Erythematosus Primary Biliary Cirrhosis Allergy Clin Immunol Pernicious Anemia Immune Thrombocytopenic Purpura 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Current Medicine Group, LLC 2009

Authors and Affiliations

  1. 1.Mount Sinai Medical CenterNew YorkUSA

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