COMMON VARIABLE IMMUNODEFICIENCY (CVID)
Sneller MC. Common variable immunodeficiency. Am J Med Sci. 2001; 321(1): 42-8.
What are the primary features of CVID?
recurrent sinopulmonary infections
• chronic diarrhea
• higher risk of malignancy, granulomatous disease, joint involvement
• Failed B cell differentiation with impaired secretion of immunoglobulins
• T cell abnormalities present as well (low CD4 count, etc) Æ therefore, this is a global immune
• More common in northern European descent (overall 1:25,000 – 1:100,000)
• Bimodal age of onset: peaks between ages 1-5 and 18-25
• Average age of onset 23-28
What infections do these patients get?
• most commonly infected with pneumococcus, hemophilus, mycoplasma
increased frequency of acute, chronic, and recurrent infections
• pneumonia, bronchitis, sinusitis, conjunctivitis, otitis
If the patient has T-cell abnormalities, they are at risk for viral (especially enterovirus) and fungal diseases
What other disorders are these patients at risk for?
• Chronic lung disease: usually in the form of bronchiectasis, can get a sarcoid-like lung disease
• Autoimmune disease: prevalence in CVID is 22%; associated with AIHA, ITP, RA, and pernicious
• Liver disease: 10% of patients will have some type of liver disease (HBV, HCV, PBC, or evidence of
• GI disease: high incidence of: inflammatory, malignant, or infectious complications, especially in patients
with T-cell deficiency (cellular immunodeficiency). Look for nodular lymphoid hyperplasia, IBD, Sprue-
like illness with flat villi, pernicious anemia, giardiasis, and malabsorption
• Lymphoma: High risk for NHL, mostly extranodal, mostly B cell type, usually well-differentiated and
secrete Ig’s; NHL may be related to the body trying to compensate for lack of immunoglobulin
• Granulomatous infiltration: mimicks sarcoidosis on pathology (non-caseating); therefor