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KEY FEATURES

  • The most common primary immunodeficiency, estimated to affect 1 in 500 individuals, although not all will be clinically symptomatic

  • Presents in adulthood

CLINICAL FINDINGS

  • Most patients are asymptomatic

  • Patients are most susceptible to infections with encapsulated bacteria (eg, Haemophilus influenzae, Streptococcus pneumoniae, Neisseria meningitides)

  • A minority of patients have frequent and recurrent sinusitis, otitis, bronchitis, or gastrointestinal infections

  • Can be associated with atopic diseases and autoimmune disorders, including Graves disease, systemic lupus erythematosus, juvenile rheumatoid arthritis, type 1 diabetes mellitus, and celiac disease

DIAGNOSIS

  • Characterized by undetectable serum IgA levels (< 7 mg/dL) with normal levels of IgG and IgM

  • Some patients with undetectable levels of serum IgA may have high titers of anti-IgA, IgG, or IgE antibodies and are at risk for anaphylactic reactions to IgA following exposure to it through infusions of plasma (or blood transfusions)

TREATMENT

  • Treatment with commercial immune globulin replacement therapy is not indicated and may result in anaphylaxis from preparations that contain detectable amounts of IgA

  • Antibiotics as needed for infections

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