IgA deficiency

Selective immunoglobulin A is a relatively mild Genetic immunodeficiency.

Iimmunoglobulin A (IgA) antibody protects against infections of the mucous membranes of the mouth, airways, and digestive tract. M

Defined as an undetectable serum IgA level in the presence of normal serum levels of IgG and IgM.

The most common of the immunodeficiencies.

Prevalence on the order of up to 1 in 333 people, making it relatively common for a genetic disease.

More common in males than in females.

Patients with selective IgA deficiency are usually asymptomatic, but can have increased frequency of infections, particularly in the respiratory, digestive and genitourinary systems.

Infections are generally mild.

May be associated with severe reactions including anaphylaxis to blood transfusions or intravenous immunoglobulin due to the presence of IgA in these blood products.

Diagnosis confirmed by laboratory measurement of IgA level in the blood.

Increased susceptibility to pneumonia and recurrent episodes of other respiratory infections and a higher risk of developing autoimmune diseases in middle age.

Similar to common variable immunodeficiency, but it does not have the same lymphocyte subpopulation abnormalities.

Patients may be susceptible to recurrent infections when on hemodialysis.[8]

Associated with autoimmune disease.

Not associated with increased risk of malignancy.

Up to 10% of IgA deficiency patients have Celiac disease.

3-5% of Celiac patients are IgA deficient.

There is no treatment for the underlying disorder.

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