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Primary Immune Deficiency Diseases

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Common Variable Immunodeficiency (CVID)

diagram of antibody immunoglobulin A
Crystal structure of the antibody immunoglobulin A (ribbon diagram)
Credit: Polish Wikipedia Project

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What is CVID?

People with CVID have low levels of immunoglobulin (antibodies) and an increased risk for infection. Although the disease is usually diagnosed in adults, it can also occur in children.

CVID is also known by the following names:

  • Hypogammaglobulinemia
  • Adult-onset agammaglobulinemia
  • Late-onset hypogammaglobulinemia
  • Acquired agammaglobulinemia

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Cause

Research has shown that CVID is more common in families with immunoglobulin A (IgA) deficiency; however, the causes of CVID have not been identified in more than 90 percent of cases.

Read about the gene linked to CVID.

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Signs and symptoms

Most people with CVID have frequent bacterial infections of the ears, sinuses, and lungs.

Some people also may have symptoms such as the following:

  • Painful swollen joints in the knee, ankle, elbow, or wrist 
  • Problems involving the digestive tract
  • Enlarged spleen and swollen glands or lymph nodes
  • Antibodies that attack their own blood cells
  • Increased risk of developing some cancers

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Diagnosis

Doctors can diagnose CVID by weighing several factors:

  • History of recurrent infections of the ears, sinuses, and lungs
  • Symptoms of digestive system problems
  • Lab tests showing very low immunoglobulin levels
  • Low antibody responses to immunization

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Treatment

People with CVID receive intravenous (through the vein) immunoglobulin every 3 to 4 weeks or subcutaneous (just under the skin) immunoglobulin weekly to restore normal antibody levels. The immunoglobulin given by either of these methods provides antibodies from about 60,000 healthy donors each time it is given. Bacterial infections are treated with antibiotics. Many patients need preventive antibiotics as well.

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Last Updated February 24, 2011

Last Reviewed October 14, 2010