Common Variable Immune Deficiency Syndromes - CVID

Common variable immune deficiency (CVID) is the most common clinically significant primary immunodeficiency disease (PID). Clinically, it is characterized by recurrent or chronic infections, especially of the sinopulmonary system, and carries an increased risk of autoimmune manifestations and malignancy. It is due to defective antibody production and hypogammaglobulinemia, with a late onset due to delayed antibody failure. There are a multitude of PIDs, and diagnosis of CVID depends on using the patient’s infection history, physical examination findings, family history, and laboratory data to differentiate it from other immune disorders. Early identification and initiation of treatment with intravenous and subcutaneous immunoglobulin can prevent major organ dysfunction.

Advances in genetic testing have made it possible to identify many monogenic forms of CVID-like conditions. Despite these advances, the genetic basis of CVID has not been completely identified, making it a diagnosis of exclusion and suggesting that it may be a polygenic condition in many patients.

Tabs Content
Content Review: 
August 2017

Last Update: November 2018