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Key Features

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

Clinical Findings

  • Most patients are asymptomatic

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

  • Frequent and recurrent sinusitis, otitis, and bronchitis

  • Can be associated with atopic diseases and autoimmune disorders, including Graves disease, SLE, 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 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 is ineffective because IgA and IgM are present only in trace quantities in these preparations

  • Antibiotics as needed for infections

  • Some cases of IgA deficiency may spontaneously remit

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