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Selective IgA deficiency: Clinical manifestations, pathophysiology, and diagnosis

Robert W Hostoffer, DO, FACOP, FAAP, FACOI, FCCP
Section Editor
E Richard Stiehm, MD
Deputy Editor
Anna M Feldweg, MD


Selective immunoglobulin A (IgA) deficiency (sIgAD) (MIM 137100) may be defined as the isolated deficiency of serum IgA (ie, in the setting of normal serum levels of immunoglobulin G [IgG] and immunoglobulin M [IgM]) in an individual older than four years of age in whom other causes of hypogammaglobulinemia have been excluded [1].

The clinical manifestations of sIgAD are variable, ranging from no symptoms to recurrent infections and autoimmune disease. This topic will review the epidemiology, clinical manifestations, diagnosis, and pathophysiology of sIgAD. The management and prognosis of patients with this disorder are discussed separately. (See "Selective IgA deficiency: Management and prognosis".)

The structure and normal functions of IgA are reviewed elsewhere. (See "Structure and biologic functions of IgA".)


Immunoglobulin A (IgA) accounts for more than 70 percent of total immunoglobulin in the body. The normal functions of IgA are mentioned briefly here and discussed in detail separately. (See "Structure and biologic functions of IgA".)

IgA exists in two distinct forms:

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Literature review current through: Oct 2017. | This topic last updated: Aug 08, 2017.
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