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Common Variable Immunodeficiency

Also called acquired hypogammaglobulinemia and agammaglobulinemia with immunoglobulin-bearing B cells, common variable immunodeficiency is characterized by progressive deterioration of B-cell (humoral) immunity. This results in increased susceptibility to infection.

Causes

Exactly what causes common variable immunodeficiency isn't known. Most patients have a normal circulating B-cell count but defective synthesis or release of immunoglobulins. Many also exhibit progressive deterioration of T-cell (cell-mediated) immunity, which is revealed by delayed hypersensitivity skin testing.

Signs and symptoms

The following are the most common symptoms of CVID. However, each child may experience symptoms differently. Symptoms may include:

• recurrent infections that can affect the ears, sinuses, and lungs (the more these infections occur, the greater the risk of scarring and permanent damage to the lungs and breathing tubes).
• inflammation in the joints of the knees, ankles, elbows, or wrists.
• stomach and bowel disorders.
• increased risk of developing some cancers.

Diagnosis

A diagnosis of CVID is usually made based on a complete medical history and physical examination. In addition, multiple blood tests may be ordered to help confirm the diagnosis.

Treatment

Patients with common variable immunodeficiency need essentially the same treatment as patients with X-linked hypogammaglobulinemia.

Injection of immune globulin (usually weekly to monthly) helps maintain immune response. Because immune globulin is composed primarily of IgG, the patient may also need fresh frozen plasma infusions to provide IgA and IgM.

Antibiotics are the mainstay for combating infection. Regular X-rays and pulmonary function studies help monitor infection in the lungs; chest physiotherapy may forestall or help clear such infection.

Prevention

The disease itself cannot be prevented, but patients and their families can take precautions to prevent the recurrent infections commonly associated with it. For example, good hygiene and nutrition are important, as is avoiding crowds or other people who have active infections.