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Selective IgA Deficiency

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Topic updated on 04/06/17 4:21pm

Snapshot
  • A 7-year-old patient presents to the emergency room after a motor vehicle accident, in which she loses a lot of blood. She is immediately transfused with blood. However, she develops difficulty breathing and oral-facial swelling. Physical exam reveals expiratory wheezes. She is immediately given epinephrine. On further questioning, physical exam reveals atopic dermatitis and recurrent ear and lung infections.
Introduction
  • Primary immunodeficiency with decreased levels of IgA
  • Epidemiology
    • most common primary immunodeficiency
  • Pathogenesis
    • B-cells fail to differentiate into IgA-producing plasma cells
    • unknown cause
    • impairs mucosal immunity, leading to sinopulmonary infections
  • Associated conditions
    • increased incidence of autoimmune diseases
    • celiac disease
    • atopy
      • allergies
      • atopic dermatitis
      • asthma
Presentation
  • Symptoms
    • most = asymptomatic
    • sinus and lung infections
    • GI infections
    • anaphylaxis with exposure to blood products with IgA
Evaluation
  • Diagnosis based on clinical history
  • Serology
    • ↓ IgA (< 7 mg/dL)
    • normal IgG, IgM, and IgG
    • false positive β-HCG
Differential
  • Common variable immunodeficiency
  • IgG deficiency
  • Ataxia-telangiectasia (↓ IgA, IgG, and IgE)
  • Hyper-IgM syndrome (↓ IgA, IgG, IgE, and ↑ IgM)
Treatment
  • Usually no specific treatment
  • Antibiotics as needed for infections
  • Immunizations
  • Blood transfusions
    • obtain blood from IgA-deficient donor
Prognosis, Prevention, and Complications
  • Prognosis
    • typically very good
    • some patients spontaneously develop normal IgA levels
  • Prevention
    • avoid blood transfusions unless from IgA-deficient individual
  • Complications
    • recurrent sinopulmonary infections
    • diarrhea from giardiasis
    • fatal anaphylaxis


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