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.
Unlike X-linked hypogammaglobulinemia (which is seen in very early childhood), this disorder usually causes symptoms after infancy and childhood, between the ages of 25 and 40. It affects men and women equally and usually doesn’t interfere with normal life span or with normal pregnancy and offspring.
Causes
Exactly what causes common variable immunodeficiency is unknown. 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
With common variable immunodeficiency, pyogenic bacterial infections are characteristic but tend to be chronic rather than acute (as in X-linked hypogammaglobulinemia). Recurrent sinopulmonary infections, chronic bacterial conjunctivitis, and malabsorption (commonly associated with infestation by Giardia lamblia) are usually the first clues to immunodeficiency.
Common variable immunodeficiency may be associated with autoimmune diseases (such as systemic lupus erythematosus, rheumatoid arthritis, hemolytic anemia, and pernicious anemia) as well as with cancers (such as leukemia and lymphoma).
Clinical tip Patients with common variable immunodeficiency can develop a nonseptic inflammatory arthritis similar to rheumatoid arthritis. However, because septic arthritis has also been reported, a search for an infecting organism should be undertaken in patients with new joint pain and inflammation, particularly if only one or two joints are affected.
Diagnosis
Characteristic diagnostic markers in this disorder include decreased serum immunoglobulin (Ig) M, IgA, and IgG detected by immunoelectrophoresis, along with a normal circulating B-cell count. Antigenic stimulation confirms an inability to produce specific antibodies; cell-mediated immunity may be intact or delayed. X-rays usually show signs of chronic lung disease or sinusitis.
Treatment
Patients with common variable immunodeficiency need essentially the same treatment as patients with X-linked hypogammaglobulinemia.
I.V. immune globulin (usually weekly to monthly) helps maintain immune response. Because immune globulin is made up 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.
Special considerations
❑ To help prevent severe infection, teach the patient and his family how to recognize its early signs. Warn them to avoid crowds and persons who have active infections.
❑ Stress the importance of good nutrition and regular follow-up care.
Book Source Details
- Book Title: Handbook of Diseases
- Author(s): Springhouse
- Year of Publication: 2003
- Copyright Details: Handbook of Diseases, Copyright © 2003 Lippincott Williams & Wilkins.
Other Book Chapters Related to Immune deficiency
Read excerpts from these other book chapters related to Immune deficiency:
Copyright Details: Handbook of Diseases, Copyright © 2008 Williams & Wilkins.
More About Causes of Immune deficiency
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More About This Book:
Title: Handbook of Diseases
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2003
ISBN: 1-58255-266-5
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» Next page: Human immunodeficiency virus infection (Handbook of Diseases)
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