Symptoms of Common Variable Immunodeficiency

Common Variable Immunodeficiency is a group of rare genetic immunodeficiency disorders. There are abnormalities in the immune cell development that result in a decreased ability to produce antibodies in response to the invasion of foreign substances. It is characterized by low levels of most or all immunoglobulin classes, lack of B lymphocytes or plasma cells that are capable of producing antibodies and frequent bacterial infections.

Common Variable Immunodeficiency is also known as Acquired Hypogammaglobulinemia, Common Variable Hypogammaglobulinemia, Late-Onset Immunoglobulin Deficiency, Acquired Agammaglobulinemia, Adult-Onset Agammaglobulinemia and Late-Onset Hypogammaglobulinemia. Common Variable Immunodeficiency shouldn't be confused with Acquired Immunodeficiency, the syndrome that is caused by the HIV virus. They differ greatly from each other.

It is more common for the onset of symptoms to occur between the second and fourth decade of life, but onset of symptoms can occur during the first decade of life. Symptoms include recurrent ear, sinus and lung infections, inflammation of the joints of the knees, ankles, elbows and wrists, stomach and bowel disorders, increased risk of developing certain cancers, regular morning cough that produces yellow or green sputum, enlarged spleen and swollen glands or lymph nodes. Gastrointestinal symptoms may include abdominal pain, bloating, nausea, vomiting, diarrhea and weight loss. Symptoms vary from one patient to another and this is why it is referred to as a variable group of disorders.

Diagnosis of Common Variable Immunodeficiency is based on a complete medical history and physical examination. Multiple blood tests, such as immune function blood tests, IgG blood test and IgA blood test, are done to help confirm the diagnosis. Alternative diagnoses that are considered during the diagnostic process include Selective IgA Deficiency, X-Linked Agammaglobulinemia, Chronic Granulomatous Disease, Hyper-IgM Syndrome, SCID and AIDS. Other underlying conditions that are related to Common Variable Immunodeficiency include Hypogammaglobulinemia, Immune Deficiency, Lymphocytopenia, Malabsorption Syndrome and Splenomegaly.

Treatment includes intravenous immunoglobulin to restore normal antibody levels, antibiotics to treat infections, physical therapy and daily postural drainage to help clear clogged lungs.

Prognosis is usually good if there is no bronchiectasis and chronic lung damage or severe autoimmune disease or malignancy. The most frequent cause of death is lymphoma. Other causes of death include liver failure, respiratory insufficiency and inflammatory bowel disease with malnutrition.

Common Variable Immunodeficiency affects all races and genders equally. Frequency is one case per 50,000 population. The exact cause is unknown. In some cases more than one person in a family may be affected, but most cases do not show a family history and there is no clear pattern of inheritance. The disorder has been associated with antirheumatic or antiepileptic drugs. Common Variable Immunodeficiency is listed as a rare disease by The Office of Rare Diseases of the National Institutes of Health because it affects less than 200,000 people in the United States Population.