Common Variable Immunodeficiency
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Questions and Answers

Which of the following is true about isolated IgA deficiency?

  • It is more common in Asian populations than Caucasian populations.
  • It is the most common primary immunodeficiency disorder. (correct)
  • The pathogenesis is well understood and involves a defect in a B-cell activating cytokine receptor.
  • Patients have normal levels of IgM, IgG, and IgE antibodies.

    • Which of the following autoimmune diseases is associated with isolated IgA deficiency?

  • Type 1 diabetes mellitus
  • Crohn's disease
  • Multiple sclerosis
  • Rheumatoid arthritis (correct)

    • Which of the following clinical features is NOT associated with isolated IgA deficiency?

  • Severe combined immunodeficiency (correct)
  • Recurrent otitis media
  • Sinusitis
  • Bronchitis

    • What is a potentially life-threatening complication of isolated IgA deficiency?

    <p>Anaphylaxis post-blood transfusion (C)</p> Signup and view all the answers

    Which of the following statements is true regarding the diagnosis of celiac disease in patients with isolated IgA deficiency?

    <p>IgA deficiency can result in false negative serology for celiac disease. (B)</p> Signup and view all the answers

    Which of the following statements is true about hyper-IgM syndrome?

    <p>It is caused by a deficiency in CD40L or lack of function. (A)</p> Signup and view all the answers

    Which of the following is a clinical feature of Severe Combined Immunodeficiency (SCID)?

    <p>Recurrent, severe infections by a wide range of pathogens (D)</p> Signup and view all the answers

    What is the most common complement deficiency mentioned in the text?

    <p>C2 deficiency (D)</p> Signup and view all the answers

    Which of the following is NOT mentioned as a potential consequence of complement deficiencies?

    <p>Isolated IgG deficiency (C)</p> Signup and view all the answers

    Which of the following is a common cause of X-linked Severe Combined Immunodeficiency (SCID)?

    <p>Mutation in the gamma-chain of various cytokine receptors (D)</p> Signup and view all the answers

    Which of the following is NOT a pathological feature of Severe Combined Immunodeficiency (SCID)?

    <p>Enlargement of lymph nodes (B)</p> Signup and view all the answers

    Which of the following autoimmune diseases is mentioned in the text as a potential complication of complement deficiencies?

    <p>Systemic lupus erythematosus (SLE) (C)</p> Signup and view all the answers

    What is the prevalence of isolated IgA deficiency?

    <p>1 in 600 (A)</p> Signup and view all the answers

    Which of the following is a common clinical feature of X-linked agammaglobulinemia (XLA)?

    <p>Recurrent respiratory infections (D)</p> Signup and view all the answers

    Which of the following is a characteristic of common variable immunodeficiency (CVID)?

    <p>All of the above (D)</p> Signup and view all the answers

    What is the primary treatment for X-linked agammaglobulinemia (XLA) and common variable immunodeficiency (CVID)?

    <p>Intravenous immunoglobulin (IVIG) (B)</p> Signup and view all the answers

    Which of the following primary immunodeficiencies is considered the rarest, based on the provided information?

    <p>Hyper IgM syndrome (C)</p> Signup and view all the answers

    Which of the following statements is true regarding the pathophysiology of X-linked agammaglobulinemia (XLA)?

    <p>All of the above (D)</p> Signup and view all the answers

    What is a common feature of common variable immunodeficiency (CVID)?

    <p>Hypogammaglobulinemia (C)</p> Signup and view all the answers

    Which of the following autoimmune diseases is commonly associated with common variable immunodeficiency (CVID)?

    <p>All of the above (D)</p> Signup and view all the answers

    Which of the following statements about the diagnosis of X-linked agammaglobulinemia (XLA) is true?

    <p>All immunoglobulins are depressed (D)</p> Signup and view all the answers

    Which of the following statements about the prognosis of X-linked agammaglobulinemia (XLA) and common variable immunodeficiency (CVID) is true?

    <p>Both a and b (B)</p> Signup and view all the answers

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