Selective deficiency of IgA
Selective deficiency of IgA is the most common immune deficiency disorder. People with this disorder have a low or absent level of a blood protein called immunoglobulin A.
Immune deficiency disorder
Immunodeficiency disorders occur when the body's immune response is reduced or absent.
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Causes
IgA deficiency is usually inherited, which means it is passed down through families. However, there are also cases of drug-induced IgA deficiency.
It may be inherited as an autosomal dominant or autosomal recessive trait. It is usually found in people of European origin. It is less common in people of other ethnicities.
Symptoms
Many people with selective IgA deficiency have no symptoms.
If a person does have symptoms, they may include frequent episodes of:
- Bronchitis (airway infection)
Bronchitis
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- Conjunctivitis (eye infection)
Conjunctivitis
The conjunctiva is a clear layer of tissue lining the eyelids and covering the white of the eye. Conjunctivitis occurs when the conjunctiva becomes ...
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Ulcerative colitis
Ulcerative colitis is a condition in which the lining of the large intestine (colon) and rectum become inflamed. It is a form of inflammatory bowel ...
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Crohn disease is a disease where parts of the digestive tract become inflamed. It most often involves the lower end of the small intestine and the be...
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Celiac disease is an autoimmune condition that damages the lining of the small intestine. This damage comes from a reaction to eating gluten. This ...
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- Otitis media (middle ear infection)
Otitis media
Suspected ear infections are one of the most common reasons parents take their children to the health care provider. The most common type of ear inf...
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- Sinusitis (sinus infection)
Sinusitis
Sinusitis is present when the tissue lining the sinuses become swollen or inflamed. It occurs as the result of an inflammatory reaction or an infect...
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- Upper respiratory tract infections
Other symptoms include:
- Bronchiectasis (a disease in which the bronchial tubes in the lungs become damaged and enlarged)
Bronchiectasis
Bronchiectasis is a disease in which the large airways in the lungs are damaged. This causes the airways to become permanently wider. Bronchiectasis...
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Exams and Tests
There may be a family history of IgA deficiency. Tests that may be done include:
- IgG subclass measurements
- Quantitative immunoglobulins
- Serum immunoelectrophoresis
Treatment
No specific treatment is available. Some people gradually develop normal levels of IgA without treatment.
Treatment involves taking steps to reduce the number and severity of infections. Antibiotics are often needed to treat bacterial infections.
Immunoglobulins are given through a vein or by injection to boost the immune system.
Autoimmune disease treatment is based on the specific problem.
Note: People with complete IgA deficiency may develop anti-IgA antibodies if given blood products and immunoglobulins. This may lead to allergies or life-threatening anaphylactic shock. However, they can safely be given IgA-depleted immunoglobulins.
Allergies
An allergy is an immune response or reaction to substances that are usually not harmful.
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Outlook (Prognosis)
Selective IgA deficiency is less harmful than many other immunodeficiency diseases.
Some people with IgA deficiency will recover on their own and produce IgA in larger quantities over a period of years.
Possible Complications
Autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, and celiac sprue may develop.
Celiac sprue
Celiac disease is an autoimmune condition that damages the lining of the small intestine. This damage comes from a reaction to eating gluten. This ...
![](../../graphics/tnail/24377t.jpg)
People with IgA deficiency may develop antibodies to IgA. As a result, they can have severe, even life-threatening reactions to transfusions of blood and blood products.
When to Contact a Medical Professional
If you have an IgA deficiency, be sure to mention it to your health care provider if immunoglobulin or other blood-component transfusions are suggested as a treatment for any condition.
Prevention
Genetic counseling may be of value to prospective parents with a family history of selective IgA deficiency.
Reviewed By
Stuart I. Henochowicz, MD, FACP, Clinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Cunningham-Rundles C. Primary immunodeficiency diseases. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 236.
Sullivan KE, Buckley RH. Primary defects of antibody production. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 150.