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Selective deficiency of IgA

Definition

Selective deficiency of IgA is the most common immune deficiency disorder. Persons with this disorder have a low or absent level of a blood protein called immunoglobulin A.

Alternative Names

IgA deficiency

Causes

IgA deficiency is usually inherited, which means it is passed down through families. However, cases of drug-induced IgA deficiency have been reported.

It may be inherited as an autosomal dominant or autosomal recessive trait. It is found in approximately 1 in 700 individuals 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
  • Chronic diarrhea
  • Conjunctivitis (eye infection)
  • Gastrointestinal inflammation, including ulcerative colitis, Crohn's disease, and a sprue-like illness
  • Mouth infection
  • Otitis media (middle ear infection)
  • Pneumonia
  • Sinusitis
  • Skin infections
  • Upper respiratory tract infections

Other symptoms include:

  • Bronchiectasis (a disease in which the small air sacs in the lungs become damaged and enlarged)
  • Unexplained asthma

Exams and Tests

There may be a family history of IgA deficiency. Tests that may be done include:

Treatment

No specific treatment is available. Some people gradually develop normal levels of IgA without treatment.

Infections should be treated with antibiotics. In some cases, longer courses of antibiotics may be needed to prevent infections from coming back.

Those with selective IgA deficiency who also have IgG subclass deficiencies may be helped with intravenous immunoglobulin (IVIG), given through a vein. Subcutaneous immunoglobulin (SCIG) treatments given under the skin may also help.

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 IVIG. This may lead to allergies or life-threatening anaphylactic shock. These individuals can safely be given IgA-depleted IVIG.

Outlook (Prognosis)

Selective IgA deficiency is less harmful than many other immunodeficiency diseases.

Some people with IgA deficiency will recover on their own and begin to 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.

Patients with IgA deficiency may develop antibodies to IgA, and can have severe, even life-threatening reactions to transfusions of blood and blood products. If transfusions are necessary, washed cells may be cautiously given.

When to Contact a Medical Professional

Consider genetic counseling if you have a family history of selective IgA deficiency and you plan to have children.

If you have an IgA deficiency, be sure to mention it to your health care provider if IVIG 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.

References

Ballow M. Primary immunodeficiency diseases. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 258.


Review Date: 5/12/2014
Reviewed By: Stuart I. Henochowicz, MD, FACP, Associate Clinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School, Washington, DC. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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