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Selective IgA deficiency: Clinical manifestations, pathophysiology, and diagnosis

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

INTRODUCTION

Selective IgA deficiency (sIgAD) (MIM 137100) may be defined as the isolated deficiency of serum immunoglobulin A (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".)

DEFINITION

Selective IgA deficiency (sIgAD) is defined as decreased serum immunoglobulin A (IgA) levels in the context of normal serum levels of immunoglobulin G (IgG) and immunoglobulin M (IgM) and in the absence of any other immunodeficiency disorder. The diagnosis should only be made after four years of age, since antibody levels in younger children can be depressed in the absence of any immune dysfunction. (See 'Diagnosis' below.)

NORMAL BIOLOGY 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".)

                                 

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