What is the role of pulmonary function testing (PFT) in the workup of agammaglobulinemia (hypogammaglobulinemia)?

Updated: Jul 08, 2019
  • Author: Donald A Person, MD, FAAP, FACR; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

The progressive nature of chronic lung disease makes pulmonary function tests (PFTs) essential in XLA. These tests include spirometry, diffusion capacity tests, and lung volume tests. They are recommended annually. Children younger than 5 years may not be able to reliably undergo these tests but some centers perform infant PFTs and/or impulse oscillometry.

PFT findings are evaluated upon diagnosis because the literature suggests that decreased parameters upon diagnosis of hypogammaglobulinemia correlate with chronic and progressive pulmonary disease such as bronchiectasis. [68] Both restrictive and obstructive patterns of chronic lung disease may occur in antibody deficiency diseases.


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