Some Types of PI:

Common Variable Immune Deficiency (CVID)

There are more than 450 different types of primary immunodeficiency (PI), and common variable immune deficiency (CVID) is a relatively common type of PI found in about 1 in 25,000 people. CVID is also known as hypogammaglobulinemia, which means low levels of immunoglobulins in the bloodstream. Because of the decreased levels of antibodies (immunoglobulins), people with CVID are prone to recurrent infections.

CVID differs from person to person. CVID symptoms, the levels of antibodies in the blood, the type of antibody that is decreased, the severity of signs and symptoms, and the time of life—childhood, adolescence, or adulthood—during which symptoms arise are all aspects that can vary.

With CVID, diagnosis often happens later than with other types of PI. So some other names have been used in the past, like adult-onset agammaglobulinemia, acquired agammaglobulinemia, and late-onset hypogammaglobulinemia.

Though the exact cause of this disorder is unknown, recent studies have shown that a small group of genes appear to be involved for some people with the disorder.

There are over 450 different types of PI, each with its own characteristics and challenges.
Need a crash course? Learn the basics of PI.

Symptoms of common variable immune deficiency (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.

CVID symptoms can include:

Recurring ear, sinus, and bronchi (breathing tubes) infections
Severe, repeated infections of the lungs (respiratory tract) that can result in permanent widening and scarring of the bronchi, a condition called bronchiectasis
Enlarged lymph nodes in the neck, chest, or abdomen
Enlarged spleen, an organ related to the immune system
Enlargement of Peyer's patches, which are collections of lymphocytes in the walls of the intestine
Collections of inflammatory cells called granuloma which can be found in the lungs, lymph nodes, liver, skin, or other organs in some cases
Painful swelling of the joints, a condition called polyarthritis, can also develop. People with CVID who develop arthritis usually experience issues in the larger joints like the knees, ankles, elbows, and wrists
Gastrointestinal complaints, such as abdominal pain, bloating, nausea, vomiting, diarrhea, and weight loss
Greater risk of cancer, especially cancers of the lymphoid system and gastrointestinal tract

Common variable immune deficiency (CVID) diagnosis

CVID testing by doctors involves measuring the titer, or concentration of antibodies in a person’s blood, shortly after giving a vaccine to determine if the immune system responds properly. A diagnosis is made when the results show low levels of antibodies in the blood, including IgG, IgA, and sometimes IgM. Special lab tests can tell whether B lymphocytes and T lymphocytes are working properly. Measuring antibody levels in the blood against vaccines such as tetanus, diphtheria, and pneumococcal polysaccharide may be another aspect of diagnosis.

“I was relieved by the diagnosis. I finally knew what I had and why I had been feeling so bad all my life.“

Katie J. Huntsville, AL

What should I know about treatment for common variable immune deficiency (CVID)?

You are not alone, but no one is quite like you. It's important to learn about the various treatments for PI, which should be individualized to meet your specific needs. Your healthcare team is there to provide recommendations based on you, and your situation. Here are some tips:

Keep track of how you’re doing and share the information with your doctor on a regular basis.
Your doctor will only know how you’re doing if you tell them. If you have questions, concerns, thoughts or feelings—speak up and share!
You and your doctor both play a part in managing your health. You can take an active role by learning as much as you can and by following your doctor’s recommendations.