Review: Autoimmunity in common variable immunodeficiency (CVID)

Common variable immunodeficiency (CVID) is the most common primary immune defect.


Humoral immunodeficiency (click to enlarge the image).

Hypogammaglobulinemia is the hallmark of CVID but other defects are also important:

- defective T-cell activation and proliferation
- dendritic cell and cytokine defects

70% to 80% of CVID patients have recurrent sinopulmonary infections. Aautoimmunity and inflammatory complications are also common.

The most common autoimmune conditions n CVID are:

- immune thrombocytopenic purpura and hemolytic anemia
- rheumatoid arthritis
- pernicious anemia
- primary biliary cirrhosis
- thyroiditis
- sicca syndrome
- systemic lupus
- inflammatory bowel disease

Treatment of autoimmunity in CVID includes high-dose immunoglobulins, corticosteroids, and selected immunosuppressants.

References:
Autoimmunity in common variable immunodeficiency. Agarwal S, Cunningham-Rundles C. Curr Allergy Asthma Rep. 2009 Sep;9(5):347-52.
Mind Maps: Primary Immunodeficiency Disorders (PIDD)