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Common Variable Immunodeficiency (CVID)

By

James Fernandez

, MD, PhD, Cleveland Clinic Lerner College of Medicine at Case Western Reserve University

Last full review/revision Apr 2021| Content last modified Apr 2021
Click here for the Professional Version
Topic Resources

Common variable immunodeficiency is an immunodeficiency disorder characterized by very low antibody (immunoglobulin) levels despite a normal number of B cells (lymphocytes).

  • People with common variable immunodeficiency may have a chronic cough, cough up blood, or have difficulty breathing (due to frequent sinus and lung infections) and may have diarrhea.

  • To diagnose the disorder, doctors measure immunoglobulin levels and determine how well the body produces immunoglobulins in response to vaccines.

  • Immune globulin is given throughout life to provide the missing immunoglobulins, and antibiotics are given to treat the frequent infections.

The genetic mutations that cause common variable immunodeficiency can be inherited, but more often, they occur spontaneously.

Symptoms of CVID

Recurring sinus and lung infections, particularly pneumonia, are common. People may develop a chronic cough, cough up blood, or have difficulty breathing. People with CVID who have frequent lung infections can develop bronchiectasis Bronchiectasis Bronchiectasis is an irreversible widening (dilation) of portions of the breathing tubes or airways (bronchi) resulting from damage to the airway wall. The most common cause is severe or repeated... read more in which the airways become widened (dilated), making it more difficult to clear mucus and to move air in and out of the lungs.

Diarrhea may occur, and food may not be absorbed well from the digestive tract. The spleen may enlarge.

Up to 25% of people develop autoimmune disorders Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers autoimmune disorders is not known. Symptoms vary depending on... read more . In autoimmune disorders, the immune system attacks the body's own tissue. Examples are autoimmune blood disorders (such as immune thrombocytopenia Immune Thrombocytopenia (ITP) Immune thrombocytopenia (ITP) is a bleeding disorder caused by decrease in the number of platelets (thrombocytes) that occurs in a person who does not have another disorder that affects platelets... read more Immune Thrombocytopenia (ITP) , autoimmune hemolytic anemia Autoimmune Hemolytic Anemia Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances... read more , and pernicious anemia Vitamin Deficiency Anemia Vitamin deficiency anemia results from low or depleted levels of vitamin B12 or folate (folic acid). People may be weak, short of breath, and pale. Nerves may malfunction. Blood tests can detect... read more ), Addison disease Addison Disease In Addison disease, the adrenal glands are underactive, resulting in a deficiency of adrenal hormones. Addison disease may be caused by an autoimmune reaction, cancer, an infection, or some... read more Addison Disease , thyroiditis Hashimoto Thyroiditis Hashimoto thyroiditis is chronic, autoimmune inflammation of the thyroid. Hashimoto thyroiditis results when the body attacks the cells of the thyroid gland—an autoimmune reaction. At first... read more , and rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more Rheumatoid Arthritis (RA) .

Most people have a normal life span, but if another disorder, such as lymphoma or an autoimmune disorder develops and is hard to treat, life span may be shortened.

Diagnosis of CVID

  • Blood tests

Doctors suspect common variable immunodeficiency when people have typical symptoms.

Blood tests are done to measure immunoglobulin levels and to determine how well the body produces immunoglobulins in response to vaccines.

If common variable immunodeficiency is diagnosed, doctors do tests yearly to check for disorders that commonly develop in people with this disorder, such as autoimmune disorders, cancers, and lung disorders. Tests may include blood tests, spirometry Pulmonary function tests measure the lungs' capacity to hold air, to move air in and out, and to absorb oxygen. Pulmonary function tests are better at detecting the general type and severity... read more (lung tests that measure how much air is inhaled and exhaled and how long each breath takes), and imaging (such as computed tomography).

Treatment of CVID

  • Immune globulin

  • Antibiotics to treat infections

Immune globulin (antibodies obtained from the blood of people with a normal immune system) is given throughout life to provide the missing immunoglobulins. It may be injected into a vein (intravenously) once a month or under the skin (subcutaneously) once a week or once a month.

Antibiotics are promptly given to treat infections. Sometimes antibiotics are taken regularly to prevent infections.

Autoimmune disorders are treated as needed with drugs that suppress or otherwise modify the immune system's activity (such as rituximab, etanercept, infliximab, or corticosteroids).

More Information about CVID

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version
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