IgA Deficiency Support Group

Selective immunoglobulin A (IgA) deficiency is a relatively mild genetic immunodeficiency. People with this deficiency lack immunoglobulin A (IgA), a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract. It is defined as an undetectable serum IgA level in the presence of normal serum levels of IgG and IgM. It is the most common of the primary antibody deficiencies.

People with selective IgA deficiency are asymptomatic, or have increased frequency of infections such as sinusitis. These infections are generally mild and would not usually lead to an in-depth workup except due to their frequency. When suspected, the diagnosis can be confirmed by laboratory measurement of IgA level. Patients have an increased susceptibility to pneumonia and recurrent episodes of other respiratory infections and a higher risk of developing autoimmune diseases in middle age.

The treatment consists of identification of comorbid conditions, preventive measures to reduce the risk of infection, and prompt and effective treatment of infections. Infections in an IgA-deficient person are treated as usual (i.e., with antibiotics). There is no treatment for the underlying disorder (except for bone marrow transplant, which is far too drastic to be used). In the absence of normal IgA, the body can develop an immune response against IgA itself, which can lead to severe reactions including anaphylaxis to blood transfusions or intravenous immunoglobulin. Therefore, people known to have this condition must notify their healthcare providers. Since this is a genetic condition, careful discussion should be undertaken prior to having children.