common variable immunodeficiency disorder CVID


Pathogenesis

  • impaired B cell differentation

Patient population

  • often has pneumonia covered by childhood vaccines. Look for encapsulated organisms (H flu, Strep pneumo)
    • rare ones include NTM, Cryptococcus
    • complement deficiency will also have encapsulated organisms but without bronchiectasis or lung diseases
  • hepatosplenomegaly
  • bilateral small lung nodules
  • bronchiectasis
  • mixed obstructive/restrictive PFT

Management

  • Check IgM, IgG, IgA 1

Footnotes

  1. Bronchiectasis Lecture by Jennifer Krall?