Understanding Common Variable Immunodeficiency (CVID)

common variable immunodeficiency

Have you been diagnosed or suspected of having Common Variable Immunodeficiency (CVID)? Did you know it’s the most frequent symptomatic primary immunodeficiency in adults? It is found in about one in 25,000 people worldwide.

What is Common Variable Immunodeficiency?

Common variable immunodeficiency (CVID) is an inherited condition of the immune system. It’s known as “primary immunodeficiency.” This is characterized by low levels of a type of protein that fights bacteria, viruses or other toxins in the body. This leads to frequent infections, particularly in the sinuses, lungs, and digestive tract.

CVID can be associated with autoimmune disorders that affect other blood cells causing low numbers of white cells or platelets, anemia, arthritis and other conditions such as endocrine disorders. In some forms of CVID, patients develop granulomas in the lungs, lymph nodes, liver, skin or other organs due to abnormal accumulation of immune cells.

While CVID is thought to be due to genetic defects, the exact cause of the disorder is unknown in the large majority of cases.

What are the Signs and Symptoms of CVID?

Some people with CVID have symptoms as young children, while many others may not develop symptoms until their 20s or 30s – or even later. It also varies widely from person to person. The most common symptoms are recurring infections involving the ears, sinuses, nose, bronchi (breathing tubes), and lungs. Severe, repeated infections of the lungs can result in permanent widening and scarring of the bronchi, a chronic condition called bronchiectasis.

Painful swelling of the joints can also develop in people with CVID. The larger joints like knees, ankles, elbows, and wrists are affected most often.

People with CVID may also complain about gastrointestinal problems such as:

  • Abdominal pain
  • Bloating
  • Nausea
  • Vomiting
  • Diarrhea
  • Weight loss

How is CVID diagnosed?

Immunologist suspects common variable immunodeficiency when a child or adult has a history of recurrent infections of the ears, sinuses, bronchi, and lungs. Individuals can be tested for CVID through laboratory tests that measure serum immunoglobulin levels. Testing for certain immune markers will indicate how well the immune system is functioning.

You may also need chest x-rays to see if CVID has caused damage to the lungs. Other tests may include biopsy and histogram to check for abnormalities and gather information on lymph nodes.

What are the Complications of CVID?

Infections that are not treated properly in people with CVID, can result in damage to organs in the body, such as the sinuses, causing chronic sinusitis, or the airways of the lung (bronchi), causing bronchiectasis.

This organ damage can lead to tissue damage, causing ongoing mucus secretion and the persistent need to clear phlegm or thick white, yellow or green mucus from the nose. Once tissue damage is established, infections become more persistent and difficult to clear.

Other complications may include:

  • Autoimmune diseases that can damage normal cells, including blood cells, skin, hair, bowel and hormone-producing glands.
  • Granulomatous disease can cause organ damage that results from immune cells which form small nodules in different tissues. These include the lungs, lymph nodes, liver, and spleen.
  • Tumors of the immune system including lymphoma may occur in some people with CVID.

How is CVID treated?

There are a variety of treatments available for CVID, the most common of which is immunoglobulin replacement therapy, which minimizes recurrent infections and reduces arthritis symptoms. This therapy is administered by intravenous or subcutaneous infusion with antibodies obtained from the fluid portion of the blood. Antibiotics, both preventatively and for treatment of specific infections, greatly improve the health and wellbeing of people with CVID by limiting the development of chronic lung disease. The dose depends on the severity of the condition.

Other treatment options include:

  • Clearance of airway secretions.
  • Corticosteroid therapy for control of autoimmune disease.
  • Management of gastrointestinal inflammation.

Affected individuals with severely low levels of circulating platelets may be cautioned to avoid the use of aspirin, since this medication may interfere with the ability of platelets to assist in the blood-clotting process. In addition, individuals with CVID should not receive live virus vaccines since there is a remote possibility that the vaccine strain of the virus may cause disease as a result of a defective immune system.

The goal of treating CVID is to control the symptoms of this disease and improve a person’s quality of life.

Takeaway

Only a certified immunologist can determine if you have common variable immunodeficiency and which treatment is right for you and your specific health needs. Visit Dr. Amy Schiffman to get tested. To request an appointment, call her at 561-409-2800.

Please call the office to discuss your allergy evaluation needs, and medications to avoid in preparation for testing.

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