Selective deficiency of IgA
IgA deficiency; Immunodepressed - IgA deficiency; Immunosuppressed - IgA deficiency; Hypogammaglobulinemia - IgA deficiency; Agammaglobulinemia - IgA deficiencySelective 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.
Read Article Now Book Mark ArticleCauses
Selective IgA deficiency is usually inherited, which means it is passed down through families. However, there are also cases of drug-induced selective 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.
Autosomal dominant
Autosomal dominant is one of many ways that a genetic trait or disorder can be passed down through families. In an autosomal dominant disease, if you...
Read Article Now Book Mark ArticleAutosomal recessive
Autosomal recessive is one of several ways that a genetic trait, disorder, or disease can be passed down through families. An autosomal recessive dis...
Read Article Now Book Mark ArticleSymptoms
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
Acute bronchitis is swelling and inflamed tissue in the bronchi, the main passages that carry air to the lungs. This swelling narrows the airways, w...
Read Article Now Book Mark Article - Chronic diarrhea
- 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 ...
Read Article Now Book Mark Article - Gastrointestinal inflammation, including ulcerative colitis, Crohn disease, and a sprue-like illness
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 ...
Read Article Now Book Mark ArticleCrohn disease
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...
Read Article Now Book Mark ArticleSprue-like illness
Celiac disease is an autoimmune condition that damages the lining of the small intestine. This damage comes from a reaction to eating gluten. This ...
Read Article Now Book Mark Article - Mouth infection
- 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...
Read Article Now Book Mark Article - Pneumonia (lung infection)
Pneumonia
Pneumonia is a breathing (respiratory) condition in which there is an infection of the lung. This article covers community-acquired pneumonia (CAP). ...
Read Article Now Book Mark Article - 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...
Read Article Now Book Mark Article - Asthma without a known cause
Asthma
Asthma is a chronic disease that causes the airways of the lungs to swell and narrow. It leads to breathing difficulty such as wheezing, shortness o...
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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
Quantitative immunoglobulins
Quantitative nephelometry is a lab test to quickly and accurately measure levels of certain proteins called immunoglobulins in the blood. Immunoglob...
Read Article Now Book Mark Article - Serum immunoelectrophoresis
Serum immunoelectrophoresis
Serum immunoelectrophoresis is a lab test that measures proteins called immunoglobulins in the blood. Immunoglobulins are proteins that function as ...
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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.
For people with more frequent or severe infections, immunoglobulins might be 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.
Read Article Now Book Mark ArticleAnaphylactic shock
Anaphylaxis is a life-threatening type of allergic reaction.
Read Article Now Book Mark ArticleOutlook (Prognosis)
Selective IgA deficiency tends to be less severe than many other immunodeficiency diseases.
Many people with selective IgA deficiency have no symptoms at all. 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 disease may develop.
Rheumatoid arthritis
Rheumatoid arthritis (RA) is a disease that leads to inflammation of the joints and surrounding tissues. It is a long-term disease. It can also aff...
Read Article Now Book Mark ArticleSystemic lupus erythematosus
Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It c...
Read Article Now Book Mark ArticleCeliac disease
Celiac disease is an autoimmune condition that damages the lining of the small intestine. This damage comes from a reaction to eating gluten. This ...
Read Article Now Book Mark ArticlePeople 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
People with IgA deficiency should contact their health care provider if they are having symptoms of an infection or intestinal symptoms.
Prevention
Genetic counseling may be of value to prospective parents with a family history of selective IgA deficiency.
References
Cunningham-Rundles C. Primary immunodeficiency diseases. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 231.
Hernandez-Trujillo VP, Ortega C. B-cell and antibody deficiencies. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 166.
Antibodies - illustration
Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.
Antibodies
illustration
Antibodies - illustration
Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.
Antibodies
illustration
Review Date: 3/31/2024
Reviewed By: Deborah Pedersen, MD, MS, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.