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Signs of Hyperinflation and Ventilation Heterogeneity in Individuals With Severe Alpha-1-Antitrypsin Deficiency at the Age of 42

Schramm, Georg Rüdiger LU ; Wollmer, Per LU ; Piitulainen, Eeva LU ; Zaigham, Suneela LU and Tanash, Hanan LU (2025) In International Journal of COPD 20. p.539-549
Abstract

Background: Severe alpha-1-antitrypsin deficiency (AATD) is a known risk factor for early development of emphysema and COPD. By the Swedish national screening program within the years 1972–74 a cohort of individuals with severe AATD (PiZZ) was identified and regularly followed up. The aim of this study was to investigate alveolar volume (VA) and the ratio VA/Total lung capacity (VA/TLC) for the detection of signs of hyperinflation and ventilation heterogeneity in PiZZ individuals compared with an age-matched control group (phenotype PiMM), randomly selected from the population registry. Methods: All study participants underwent pulmonary function tests (PFT) including dynamic spirometry and total lung... (More)

Background: Severe alpha-1-antitrypsin deficiency (AATD) is a known risk factor for early development of emphysema and COPD. By the Swedish national screening program within the years 1972–74 a cohort of individuals with severe AATD (PiZZ) was identified and regularly followed up. The aim of this study was to investigate alveolar volume (VA) and the ratio VA/Total lung capacity (VA/TLC) for the detection of signs of hyperinflation and ventilation heterogeneity in PiZZ individuals compared with an age-matched control group (phenotype PiMM), randomly selected from the population registry. Methods: All study participants underwent pulmonary function tests (PFT) including dynamic spirometry and total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC) and alveolar volume (VA). They answered a questionnaire on smoking habits and symptoms. Quality of life was assessed by answering the Saint George´s Respiratory Questionnaire (SGRQ). Results: Fifty-six PiZZ and 66 PiMM individuals participated in the study. The PiZZ individuals had a significantly lower VA/TLC ratio (p=0.004) and significantly higher RV (p<0.001) and RV/TLC ratio (p=0.001) than the PiMM individuals. The 13 PiZZ ever-smokers had a significantly higher TLC (p=0.025), RV (p=0.003) and FRC (p=0.003) and a significantly lower VA/TLC ratio (p=0.006) than the 24 PiMM ever-smokers. No significant differences in RV, TLC, VA, and VA/TLC ratio were found between the never-smoking PiZZ and PiMM individuals. The ratio RV/TLC was significantly higher in the PiZZ never-smokers than in the PiMM never-smokers (p=0.026). Conclusion: At the age of 42 the PiZZ ever-smokers have signs of hyperinflation and ventilation heterogeneity.

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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
alpha-1-antitrypsin-deficiency, alveolar volume, hyperinflation, smoking
in
International Journal of COPD
volume
20
pages
11 pages
publisher
Dove Medical Press Ltd.
external identifiers
  • pmid:40066198
  • scopus:86000773706
ISSN
1176-9106
DOI
10.2147/COPD.S486575
language
English
LU publication?
yes
id
ea3ad663-d49a-48d9-b85d-a467406ca019
date added to LUP
2025-06-25 09:05:35
date last changed
2025-07-09 11:17:34
@article{ea3ad663-d49a-48d9-b85d-a467406ca019,
  abstract     = {{<p>Background: Severe alpha-1-antitrypsin deficiency (AATD) is a known risk factor for early development of emphysema and COPD. By the Swedish national screening program within the years 1972–74 a cohort of individuals with severe AATD (PiZZ) was identified and regularly followed up. The aim of this study was to investigate alveolar volume (V<sub>A</sub>) and the ratio V<sub>A</sub>/Total lung capacity (V<sub>A</sub>/TLC) for the detection of signs of hyperinflation and ventilation heterogeneity in PiZZ individuals compared with an age-matched control group (phenotype PiMM), randomly selected from the population registry. Methods: All study participants underwent pulmonary function tests (PFT) including dynamic spirometry and total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC) and alveolar volume (V<sub>A</sub>). They answered a questionnaire on smoking habits and symptoms. Quality of life was assessed by answering the Saint George´s Respiratory Questionnaire (SGRQ). Results: Fifty-six PiZZ and 66 PiMM individuals participated in the study. The PiZZ individuals had a significantly lower V<sub>A</sub>/TLC ratio (p=0.004) and significantly higher RV (p&lt;0.001) and RV/TLC ratio (p=0.001) than the PiMM individuals. The 13 PiZZ ever-smokers had a significantly higher TLC (p=0.025), RV (p=0.003) and FRC (p=0.003) and a significantly lower V<sub>A</sub>/TLC ratio (p=0.006) than the 24 PiMM ever-smokers. No significant differences in RV, TLC, V<sub>A</sub>, and V<sub>A</sub>/TLC ratio were found between the never-smoking PiZZ and PiMM individuals. The ratio RV/TLC was significantly higher in the PiZZ never-smokers than in the PiMM never-smokers (p=0.026). Conclusion: At the age of 42 the PiZZ ever-smokers have signs of hyperinflation and ventilation heterogeneity.</p>}},
  author       = {{Schramm, Georg Rüdiger and Wollmer, Per and Piitulainen, Eeva and Zaigham, Suneela and Tanash, Hanan}},
  issn         = {{1176-9106}},
  keywords     = {{alpha-1-antitrypsin-deficiency; alveolar volume; hyperinflation; smoking}},
  language     = {{eng}},
  pages        = {{539--549}},
  publisher    = {{Dove Medical Press Ltd.}},
  series       = {{International Journal of COPD}},
  title        = {{Signs of Hyperinflation and Ventilation Heterogeneity in Individuals With Severe Alpha-1-Antitrypsin Deficiency at the Age of 42}},
  url          = {{http://dx.doi.org/10.2147/COPD.S486575}},
  doi          = {{10.2147/COPD.S486575}},
  volume       = {{20}},
  year         = {{2025}},
}