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
- sicca syndrome
- systemic lupus
- inflammatory bowel disease
Treatment of autoimmunity in CVID includes high-dose immunoglobulins, corticosteroids, and selected immunosuppressants.
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)