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Your immune system is made up of various cells and proteins that work together to fight illness-causing germs like viruses and bacteria. One of these proteins is immunoglobulin A (IgA), an antibody found mainly in areas that come into contact with the outside world, including the gastrointestinal (GI) tract, respiratory tract, and urinary tract.
Selective IgA deficiency means your body makes little or no IgA, while other antibody levels stay normal. This can put you at risk of frequent infections, though many people with a selective IgA deficiency don’t realize they have it.
In this article, we’ll cover everything you should know about selective IgA deficiency and what you can do to manage it.
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What Is Selective IgA Deficiency? Selective IgA deficiency is the most common type of primary immunodeficiency — also called inborn errors of immunity (IEI) — in North America, affecting around 1 in 500 people.
Because IgA functions as part of your immune system, having selective IgA deficiency may increase the risk of:
Although your blood doesn’t have detectable amounts of IgA, the rest of your immune system likely functions normally. This is why many people don’t know they have an IgA deficiency unless a blood test detects it.
The majority of people with a selective IgA deficiency have no symptoms. If you have a selective IgA deficiency, you might get sick more often than your friends, family, or co-workers, but it might not be often enough to stand out as a health concern.
The main symptoms of a selective IgA deficiency are recurrent infections, especially involving areas where IgA is most active within the immune system. These include the GI tract and respiratory system.
Recurrent sinus infections are among the most common infections in people with a selective IgA deficiency. A sinus infection affects the air-filled cavities around your nose, cheeks, and forehead, which usually produce mucus to help clear your nose of germs and allergens.
Sinus infections cause symptoms like a stuffy nose, runny nose, or mucus dripping down your throat. You might also get headaches or feel a lot of pressure in your face and head.
Recurrent ear infections are also common in people with a selective IgA deficiency. An ear infection can happen if viruses or bacteria infiltrate the fluid behind your inner ear and cause inflammation. This can result in ear pain, pressure, muted hearing, and sometimes ear drainage.
Bronchitis and pneumonia are two common respiratory infections that affect some people with selective IgA deficiency repeatedly. Chronic or recurrent respiratory infections can cause a persistent cough, tightness in the chest, or wheezing when you breathe.
You may experience repeated infections involving the gut or stomach, including a parasitic infection called giardiasis. If your selective IgA deficiency involves your GI tract, you may experience symptoms like chronic diarrhea or belly pain.
IgA deficiency can also increase your risk of other conditions that involve the immune system, including the following.
Allergies and asthma are closely linked, and they frequently appear in people with a selective IgA deficiency. Allergies occur when your immune system is overly sensitive to something that isn’t typically harmful to other people.
If you have symptoms of allergic reactions, such as itchy eyes, skin reactions, or asthma attacks, your doctor may use allergy testing to confirm an allergy. Asthma symptoms, like trouble breathing, may warrant lung function tests.
Rheumatoid arthritis, an autoimmune condition that causes joint pain and stiffness, is also one of the most common autoimmune diseases that co-occurs with selective IgA deficiency.
Selective IgA deficiency can increase your risk of inflammatory bowel disease. This can cause, contribute to, or worsen chronic diarrhea.
Health experts don’t know why some people aren’t able to produce enough immunoglobulin A, or why some people don’t produce any IgA. However, there are some risk factors linked to this condition:
Health experts estimate that around 20 percent of selective IgA deficiency cases are inherited, according to the Immune Deficiency Foundation. You may also be at an increased risk of the disease if you have family members with other primary immunodeficiency conditions.
Research shows that families with cases of common variable immunodeficiency are also susceptible to selective IgA deficiencies. Furthermore, some people with selective IgA deficiency are later diagnosed with CVID.
Some medications can directly cause a selective IgA deficiency, and that deficiency can persist even after you stop taking the medication. Medications linked to selective IgA deficiency include drugs that treat epilepsy, seizures, and rheumatoid arthritis.
Only a blood test can confirm the diagnosis. The blood test measures the level of IgA in your blood — it confirms a complete deficiency if you have no IgA in your blood or a partial deficiency if you have low levels of IgA.
Your doctor may recommend an immunoglobulin A blood test if you keep getting sick, whether it’s the same illness repeatedly or different illnesses commonly linked to a selective IgA deficiency. In the process, they might use other diagnostic tests to rule out other possible causes of recurrent illnesses.
If selective IgA deficiency doesn’t cause symptoms or complications, you may not need treatment. Care usually focuses on preventing or treating infections.
Doctors prescribe antibiotics to treat recurrent bacterial infections in people with selective IgA deficiency. The exact germ causing your infection might not be detectable, so your doctor may prescribe a broad-spectrum antibiotic that can treat multiple infections.
If you experience chronic infections such as chronic bronchitis or chronic sinusitis, your doctor may prescribe preventive antibiotics to help prevent flare-ups.
Staying up to date on vaccinations may help prevent some infections. Ask your doctor which vaccines are right for you.
For some people, having a selective IgA deficiency can come with safety risks. More specifically, you may be at risk of having a negative reaction to blood products during a blood transfusion. If you need a blood transfusion, your healthcare provider should ensure you receive washed or IgA-poor red blood cells.
Your doctor may refer you to an immunologist after diagnosing you with a selective IgA deficiency. An immunologist can treat immune system problems associated with your deficiency, including allergies and asthma.
If you are experiencing symptoms suggestive of an autoimmune condition, such as rheumatoid arthritis, your doctor may refer you to a rheumatologist.
Tell your doctor if you experience any new symptoms that may be due to an allergic reaction or another related condition.
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 selective IgA deficiency? Let others know in the comments below.
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