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Updated: Dec 25 2021

IgA Deficiency

  • Snapshot
    • A 22-year-old female with a history of celiac disease comes to the office for chronic watery diarrhea. She recently returned from a vacation in Mexico. On further chart review, she has had multiple episodes of sinus infections, pneumonia, and otitis media throughout her childhood. After her last pregnancy, she received a blood transfusion following severe post-partum hemorrhage and experienced an anaphylactic reaction. Physical exam reveals chronic eczema on the flexural surface of the arms. Stool analysis is positive for Giardia.
  • Introduction
    • Primary immunodeficiency with decreased levels of IgA
  • Epidemiology
    • Most common primary immunodeficiency
    • May be inherited (recessive and dominant forms identified)
    • May be drug-induced
    • Most common in individuals of European descent
    • Prevalence 1 in 500 persons
    • Pathogenesis
      • cause is unknown
      • B-cells fail to differentiate into IgA producing plasma cells
      • 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
        • usually Streptococcus pneumoniae and other encapsulated bacteria
      • GI infections
        • especially giardiasis
      • severe allergies
      • atopic dermatitis
      • anaphylaxis with exposure to blood products containing IgA
    • Radiography
      • to identify infections secondary to low IgA
    • Serology
      • ↓ IgA (< 7 mg/dL)
      • normal IgG and IgM
      • false positive β-hCG
  • Differential Diagnosis
    • Common variable immunodeficiency
    • IgG deficiency
    • Ataxia-telangiectasia (↓ IgA, IgG, and IgE)
    • Hyper-IgM syndrome (↓ IgA, IgG, IgE, and ↑ IgM)
      • normal B and T cell by flow cytometry
        • may have deficient expression of CD40L on activated T-helper cells
      • presents with
        • severe pyogenic infections early in life
        • chronic diarrhea
        • opportunistic infections, such as recurrent pneumonia
      • increased risk for
        • hyperviscosity syndrome
          • due to elevated levels of IgM
        • malignancies (HCC and carcinoid tumor)
    • Diagnosis based on clinical history
  • Treatment
    • Prevention
      • avoid blood transfusions unless treated with saline wash or from IgA deficient individual
    • Usually no specific treatment
    • Antibiotics as needed for infections
    • Immunizations
    • Blood transfusions
      • wash RBCs with saline or
      • obtain blood from IgA-deficient donor
  • Complications
    • Recurrent sinopulmonary infections
    • Diarrhea from Giardiasis
    • Fatal anaphylaxis
  • Prognosis
    • Typically very good
    • Some patients spontaneously develop normal IgA levels
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