Please confirm topic selection

Are you sure you want to trigger topic in your Anconeus AI algorithm?

Please confirm action

You are done for today with this topic.

Would you like to start learning session with this topic items scheduled for future?

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
1 of 0
1 of 4
Private Note

Attach Treatment Poll
Treatment poll is required to gain more useful feedback from members.
Please enter Question Text
Please enter at least 2 unique options
Please enter at least 2 unique options
Please enter at least 2 unique options