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Lung function trajectories in common variable immunodeficiencies: An observational retrospective multicenter study.

Helena Buso1, Davide Firinu2, Renato Finco Gambier1

  • 1Department of Medicine, DIMED, University of Padova, Padova, Italy; Internal Medicine 1, Ca' Foncello University Hospital, AULSS2, Treviso, Italy.

The Journal of Allergy and Clinical Immunology
|November 20, 2024
PubMed
Summary
This summary is machine-generated.

Patients with common variable immunodeficiencies (CVIDs) often have reduced lung function, but their lung decline is not accelerated after diagnosis. This study analyzed lung physiology in CVIDs, finding most had lower lung volumes but a stable decline rate.

Keywords:
B cell subtypesCommon variable immunodeficienciesGlobal Lung Function Initiative (GLI)bronchiectasischronic obstructive pulmonary disease (COPD)granulomatous lymphocytic interstitial lung disease (GLILD)pulmonary function tests

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Area of Science:

  • Pulmonology
  • Immunology
  • Internal Medicine

Background:

  • Respiratory disease is a significant cause of illness and death in common variable immunodeficiencies (CVIDs).
  • Lung function trajectories in CVIDs are not well understood, necessitating further investigation.

Purpose of the Study:

  • To determine lung physiology measurements in patients with CVIDs.
  • To analyze the temporal trajectory of lung function in CVIDs.
  • To investigate the association between lung function and clinical/immunologic parameters in CVIDs.

Main Methods:

  • Retrospective analysis of longitudinal pulmonary function tests (PFTs) and chest CT scans from 185 CVID patients across 5 Italian centers.
  • PFTs were standardized using the European Respiratory Society/American Thoracic Society 2021 guidelines, expressing results as percentiles within a healthy population's normal distribution.
  • Investigated associations between lung function parameters (FEV1, FVC) and clinical/immunologic factors, including switched-memory B cell counts.

Main Results:

  • The majority of CVID patients exhibited lung volumes in the lower third of the normal distribution; 23% had FEV1 < lower limit of normal (LLN) and 21% had FVC < LLN.
  • Low switched-memory B cells (<2%) were significantly associated with reduced FEV1 (OR 7.58) and FVC (OR 3.55).
  • In patients with at least 5 years of PFT data, the annual decline in FEV1 (25.6 mL/year) and FVC (15.6 mL/year) did not differ significantly from predicted rates.

Conclusions:

  • Most patients with CVIDs present with lung volumes below the median of healthy individuals.
  • The rate of lung function decline in CVID patients is not accelerated post-diagnosis, suggesting a stable disease course in terms of pulmonary function.
  • Identifying immunologic markers like switched-memory B cells may help predict lung function impairment in CVIDs.