Common variable immunodeficiency (CVID) is a disorder of the immune system that can leave you more vulnerable to infections. Formerly known as adult-onset hypogammaglobulinemia, CVID is one of the most common types of primary immunodeficiency and affects roughly 1 in 25,000 people.

Want to know more about CVID, common CVID symptoms, and treatments that help manage it? This article will give you basic information you should know about CVID and its underlying causes.

What Is CVID?

Common variable immunodeficiency is a primary immunodeficiency disorder that causes your body to produce low levels of certain antibodies. Antibodies are proteins within the immune system that latch onto unwanted substances (like viruses or bacteria) so that your immune system can remove them from your body before they make you sick.

When your immune system doesn’t produce enough antibodies, like in CVID, you have an increased risk of recurrent infections and are less responsive to vaccines. Vaccines won’t protect you as well as they would for someone else because your body can’t create antibodies in response to them.

🗳️ How often do you get infections with common variable immunodeficiency (CVID)?

Several times a year

Once or twice a year

Less than once a year

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Common Variable Immunodeficiency Symptoms

Most people with CVID are first diagnosed in adulthood, but CVID symptoms may have affected you since childhood. The main symptom of CVID is frequent infections — you might often get sick with ear infections, sinus infections, and other common illnesses.

Frequent infections are a key feature of CVID, but they’re not the only symptoms. When you have CVID, you can also experience day-to-day symptoms, including the following.

Ongoing Gastrointestinal Symptoms

Roughly 21 percent of people with CVID have chronic or long-lasting gastrointestinal (GI, or digestive tract) symptoms, including diarrhea, nausea, vomiting, and bloating. These issues may happen because of a reduced ability to absorb fats and sugars from your diet or from GI inflammation.

Unintentional Weight Loss

Chronic (long-term) GI problems like diarrhea and malabsorption (difficulty absorbing nutrients from food) can lead to unintentional weight loss. You might have a hard time gaining any weight if you’ve been told you’re underweight.

Runny Nose

CVID can cause you to have a runny nose all the time, which is called chronic rhinitis. You might feel like your nose is always “stuffed” and have a hard time breathing because of it.

Fatigue

People with CVID often experience fatigue or tiredness that doesn’t improve with rest or sleep. In one study of people with CVID, almost 77 percent reported fatigue as a symptom, which significantly reduces quality of life.

Swollen Lymph Nodes

Lymph nodes are small, bean-shaped structures that help filter germs and other substances from lymph fluid. In most people, swollen lymph nodes are a sign of an immune response to an infection.

If you have CVID, your lymph nodes may be chronically enlarged because of immune cells gathering abnormally in them. You may be able to feel swollen lymph nodes in your neck, armpits, or groin area.

Long-Term Risks of CVID

Following are some serious complications that can occur with CVID because of organ damage from infections or reduced immunity.

Autoimmune Diseases

Roughly one-quarter of people with CVID develop an autoimmune disease, which occurs when your immune system starts targeting otherwise healthy organs and tissues.

The most common autoimmune diseases in people with CVID are blood-related autoimmune conditions like immune thrombocytopenia.

Chronic Lung Problems

CVID can increase your risk of chronic respiratory problems, such as bronchiectasis, in which damaged airways become permanently widened and have difficulty clearing mucus and moving air in and out.

Chronic lung diseases can occur because of repeated respiratory infections, such as pneumonia and bronchitis.

Enlarged Spleen

Your spleen is a small organ in your abdomen that helps filter your blood as part of your immune system. Your spleen may be chronically enlarged or swollen if you have CVID because of immune cells accumulating in the organ.

Increased Cancer Risk

CVID can increase your risk of certain types of cancer, including non-Hodgkin lymphoma (a cancer of the immune system). CVID can also increase the risk of stomach cancer, but this is less common.

CVID Causes

Genetic mutations (variations) are linked to some cases of CVID, but the underlying cause is unknown for most people. In about 10 percent of cases, doctors can identify an inherited genetic variation, a change passed down from a parent, that contributes to the condition. In the other 90 percent of cases, health experts haven’t identified a specific cause.

CVID isn’t associated with just one gene change — there are many gene variations related to the condition, and most people with CVID have multiple gene variations linked to CVID. These gene variations cause B cells within your immune system to function improperly. Functioning B cells produce antibodies to help protect you from illnesses.

How CVID Is Diagnosed

If you frequently get infections, or if you have a poor response to vaccines, your doctor will ask about your symptoms and medical history. Based on the information they gather, they may recommend testing for CVID.

To diagnose CVID, doctors collect a blood sample. They measure the amount of antibodies in the blood sample. If testing shows low immunoglobulin levels and a poor antibody response to vaccines, your doctor will also rule out other possible causes of antibody deficiency. Genetic testing may be considered in some cases, but it is not required to diagnose CVID.

However, many people are diagnosed with CVID based on their symptoms and lab tests alone, and no genetic variations are found as a cause of the condition.

After a CVID diagnosis has been confirmed, your doctor may recommend yearly tests to check for CVID complications like chronic lung diseases and autoimmune disorders. They may recommend spirometry (lung function tests) or imaging tests among other screenings.

Treatment and Management of CVID

While living with CVID, you’ll work closely with your healthcare team to reduce the frequency of infections, manage them as they occur, and screen for complications.

CVID Treatment

CVID is usually treated with lifelong immunoglobulin replacement therapy. This treatment gives your body antibodies it can’t make on its own. You and your doctor can choose the option that works best for you:

• Intravenous immunoglobulin (IVIg) is given through a vein, typically every three to four weeks.
• Subcutaneous immunoglobulin (SCIg) is given as shots under the skin, typically every one to four weeks.

Infection Prevention and Management

Along with immunoglobulin replacement therapy, your doctor may recommend other steps to reduce your risk of infections. For example, they might prescribe preventive antibiotics to help stop certain bacterial infections before they develop.

Vaccination recommendations vary for people with CVID. Nonlive vaccines are generally considered safe, although they may not produce as strong an immune response as they do in people without CVID.

Some live vaccines may be unsafe or ineffective, particularly for people with certain immune-system abnormalities or those receiving immunoglobulin replacement therapy.

Your doctor can tell you which vaccines are appropriate based on your immune function, treatment plan, age, and medical history. These may include nonlive vaccines for illnesses such as influenza, COVID-19, tetanus, or shingles.

Vaccines may help prevent infections or reduce their severity, but the level of protection can vary from person to person.

Lifestyle Management

Your doctor might advise you to make mindful lifestyle changes in an effort to prevent infections and CVID complications. It can help to:

• Avoid contact with people who you know are sick with contagious illnesses.
• Wear a mask while in public.
• Wash your hands frequently.
• Use hand sanitizer when water and soap aren’t available.
• Avoid smoke exposure and other fumes that can irritate your lungs.

When To Seek Urgent Care

There may be times that you need urgent care while living with CVID. Get in touch with your doctor or visit your nearest urgent care center right away if you notice signs of possible CVID complications, including:

• Difficulty breathing
• Coughing up blood
• A fever, especially with joint pain or diarrhea
• Mental confusion or vertigo
• Signs of dehydration from diarrhea

Join the Conversation

On myPIteam, people share their experiences with primary immunodeficiency, get advice, and find support from others who understand.

What can you share about your experience with common variable immunodeficiency? Let others know in the comments below.