Common Variable Immunodeficiency (CVID)

Overview

What is common variable immunodeficiency (CVID)?

CVID is a genetic disorder that affects the immune system. People with this condition have low levels of antibodies (proteins that fight infections) in their blood. When the body does not have enough of these antibodies, people may experience frequent infections.

In people with CVID, infections often develop in the respiratory system, ears and sinuses. CVID can increase your risk of developing digestive problems and cancer.

CVID is a primary immunodeficiency disease (PIDD). These diseases are genetic disorders in which a person’s immune system does not work properly.

Who is affected by common variable immunodeficiency (CVID)?

CVID occurs in about 1 out of 25,000 people. It affects males and females in equal numbers. The condition can appear in children or teens, but usually isn’t recognized until adulthood. In most cases, a doctor diagnoses the disorder when a person is between the ages of 20 to 50.

Symptoms and Causes

What causes common variable immunodeficiency (CVID)?

A genetic mutation (change) causes CVID. In most cases, this genetic mutation — and CVID symptoms — develop without any apparent cause. In about 10 percent of cases, CVID is hereditary (passed down among family members).

CVID results from defects in the genes involved with the immune system. These defects cause the body to produce abnormally low amounts of a proteins called immunoglobulins, including immunoglobulin G (IgG). Low levels of IgG in the blood can make it difficult for the body to fight infections.

What are the symptoms of common variable immunodeficiency (CVID)?

Symptoms of CVID vary widely from person to person. They can range from mild to severe. Signs and symptoms of CVID include:

  • Breathing problems
  • Chronic cough
  • Diarrhea that causes weight loss
  • Ear infections
  • Frequent sinus infections
  • Recurring lung infections, including pneumonia

Diagnosis and Tests

How is common variable immunodeficiency (CVID) diagnosed?

A doctor might look into CVID as a possible cause if you have signs of an immune disorder or have unusual reactions to vaccines.

Your doctor will ask about your family medical history and order blood tests. A sample of your blood can reveal signs of CVID, including low levels of IgG.

Blood tests can also help your doctor evaluate how well your immune system functions. Your doctor may give you a vaccine for another condition to see how your body responds to the vaccine.

Management and Treatment

What are the treatments for common variable immunodeficiency (CVID)?

If a diagnosis of CVID is made, a doctor will prescribe immunoglobulin (IgG) replacement therapy to try to prevent infections. People who need IgG therapy have it throughout their whole lives — it’s not a one-time treatment.

IgG therapy replaces your missing immunoglobulin with antibodies from the blood of a pool of healthy donors. You’ll receive this treatment intravenously (through a needle inserted into the vein) or by an injection under the skin (subcutaneous).

Your doctor may also prescribe medications called antibiotics to treat infections related to CVID, and other medications to manage complications of CVID.

What are the complications associated with common variable immunodeficiency (CVID)?

People with CVID have an increased risk of developing complications including:

  • Autoimmune disorders: Conditions where the body’s immune system harms its own cells. Some people with CVID develop immune thrombocytopenia (a low level of platelets in the blood) or autoimmune hemolytic anemia (when the body’s immune system destroys red blood cells).
  • Bronchiectasis: Permanent damage to the lungs from recurrent infections.
  • Cancers: Conditions including lymphoma and stomach cancer.
  • Granulomas: Inflamed cells in the skin, lungs, and other organs.

Prevention

How can you prevent common variable immunodeficiency (CVID)?

You cannot prevent CVID. This condition is passed down through a family (genetic disorder) and is present throughout a person’s life.

Who is at risk of developing common variable immunodeficiency (CVID)?

In a small number of cases, people who have a family member with CVID are at a higher risk of developing the disorder. In most cases, doctors do not know what causes the gene mutation that causes CVID.

Outlook / Prognosis

What is the prognosis (outlook) for people with common variable immunodeficiency (CVID)?

CVID has no cure. With ongoing treatment, many people with the disorder live active and fulfilling lives.

In some cases, complications of CVID such as lung damage or cancer may affect life expectancy. These complications appear over time. They may become life-threatening, but that process often takes years.

Living With

When should I see a healthcare provider about common variable immunodeficiency (CVID)?

Contact your healthcare provider if you have frequent or repeated infections or other symptoms of CVID.

What questions should I ask my doctor?

If you have common variable immunodeficiency, you may want to ask your doctor:

  • How serious is CVID?
  • What caused this condition?
  • How will it affect my home and work life?
  • What signs of complications should I look out for?

Last reviewed by a Cleveland Clinic medical professional on 07/08/2019.

References

  • American Academy of Allergy Asthma & Immunology. Common variable immunodeficiency (CVID). (https://www.aaaai.org/conditions-and-treatments/primary-immunodeficiency-disease/common-variable-immunodeficiency) Accessed 7/9/2019.
  • US National Library of Medicine, Genetics Home Reference. Common variable immune deficiency. (https://ghr.nlm.nih.gov/condition/common-variable-immune-deficiency) Accessed 7/9/2019.
  • Immune Deficiency Foundation. CVID Community Center. (https://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/common-variable-immune-deficiency/) Accessed 7/9/2019.
  • Merck Manual. Common Variable Immunodeficiency (CVID). (https://www.merckmanuals.com/home/immune-disorders/immunodeficiency-disorders/common-variable-immunodeficiency-cvid) Accessed 7/9/2019.
  • National Organization for Rare Disorders. Common Variable Immune Deficiency. (https://rarediseases.org/rare-diseases/common-variable-immune-deficiency/) Accessed 7/9/2019.

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