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Lung Function and Health Status in Individuals with Severe Alpha-1-Antitrypsin Deficiency at the Age of 42

Schramm, Georg Rüdiger LU ; Mostafavi, Behrouz LU ; Piitulainen, Eeva LU ; Wollmer, Per LU and Tanash, Hanan A. LU (2021) In International Journal of COPD 16. p.3477-3485
Abstract

Background: Severe hereditary alpha-1-antitrypsin deficiency (AATD) is a known risk factor for the early development of pulmonary emphysema and COPD, especially in smo-kers. By the Swedish national screening programme carried out from 1972 to 1974, a cohort of individuals with severe (PiZZ) AATD was identified and has been followed up regularly. The aim of this study was to investigate health status, quality of life and lung function in this cohort at the age of 42 years compared with an age-matched control group randomly selected from the population registry. Methods: All study participants answered a questionnaire on smoking habits, symptoms, occupation, exposure to airway irritants and quality of life using Saint George’s Respiratory... (More)

Background: Severe hereditary alpha-1-antitrypsin deficiency (AATD) is a known risk factor for the early development of pulmonary emphysema and COPD, especially in smo-kers. By the Swedish national screening programme carried out from 1972 to 1974, a cohort of individuals with severe (PiZZ) AATD was identified and has been followed up regularly. The aim of this study was to investigate health status, quality of life and lung function in this cohort at the age of 42 years compared with an age-matched control group randomly selected from the population registry. Methods: All study participants answered a questionnaire on smoking habits, symptoms, occupation, exposure to airway irritants and quality of life using Saint George’s Respiratory Questionnaire (SGRQ). They underwent complete pulmonary function tests (PFT) and forced oscillation technique (FOT) for the measurement of airway resistance and reactance. Blood samples were taken for allergies and IgG-subclasses as an indicator of increased risk of airway infections. Results: The residual volume (RV), total lung capacity (TLC) and RV/TLC ratio were significantly higher in the PiZZ ever-smokers compared to the PiMM ever-smokers and PiZZ never-smokers (p < 0.05). The resistance in the upper, small and total airways was significantly lower in PiZZ subjects compared to PiMM subjects (p < 0.05). A greater propor-tion of PiZZ never-smokers had an FEV1 /VC ratio <0.7 than PiMM never-smokers (p = 0.043). PiZZ subjects with occupational exposure to airway irritants showed a significantly lower FEV1, VC and higher RV/TLC ratio than PiMM individuals with exposure (p < 0.05). Conclusion: At the age of 42, ever-smoking PiZZ individuals have signs of COPD, and also PiZZ never-smokers have early, physiological signs of emphysema.

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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Alpha-1-antitrypsin deficiency, COPD, Lung function, Quality of life
in
International Journal of COPD
volume
16
pages
9 pages
publisher
Dove Medical Press Ltd.
external identifiers
  • pmid:34992356
  • scopus:85123270453
ISSN
1176-9106
DOI
10.2147/COPD.S335683
language
English
LU publication?
yes
id
5318d48b-a057-4b4d-a994-532d09f0797a
date added to LUP
2022-03-23 13:46:51
date last changed
2024-07-06 22:36:57
@article{5318d48b-a057-4b4d-a994-532d09f0797a,
  abstract     = {{<p>Background: Severe hereditary alpha-1-antitrypsin deficiency (AATD) is a known risk factor for the early development of pulmonary emphysema and COPD, especially in smo-kers. By the Swedish national screening programme carried out from 1972 to 1974, a cohort of individuals with severe (PiZZ) AATD was identified and has been followed up regularly. The aim of this study was to investigate health status, quality of life and lung function in this cohort at the age of 42 years compared with an age-matched control group randomly selected from the population registry. Methods: All study participants answered a questionnaire on smoking habits, symptoms, occupation, exposure to airway irritants and quality of life using Saint George’s Respiratory Questionnaire (SGRQ). They underwent complete pulmonary function tests (PFT) and forced oscillation technique (FOT) for the measurement of airway resistance and reactance. Blood samples were taken for allergies and IgG-subclasses as an indicator of increased risk of airway infections. Results: The residual volume (RV), total lung capacity (TLC) and RV/TLC ratio were significantly higher in the PiZZ ever-smokers compared to the PiMM ever-smokers and PiZZ never-smokers (p &lt; 0.05). The resistance in the upper, small and total airways was significantly lower in PiZZ subjects compared to PiMM subjects (p &lt; 0.05). A greater propor-tion of PiZZ never-smokers had an FEV<sub>1</sub> /VC ratio &lt;0.7 than PiMM never-smokers (p = 0.043). PiZZ subjects with occupational exposure to airway irritants showed a significantly lower FEV<sub>1</sub>, VC and higher RV/TLC ratio than PiMM individuals with exposure (p &lt; 0.05). Conclusion: At the age of 42, ever-smoking PiZZ individuals have signs of COPD, and also PiZZ never-smokers have early, physiological signs of emphysema.</p>}},
  author       = {{Schramm, Georg Rüdiger and Mostafavi, Behrouz and Piitulainen, Eeva and Wollmer, Per and Tanash, Hanan A.}},
  issn         = {{1176-9106}},
  keywords     = {{Alpha-1-antitrypsin deficiency; COPD; Lung function; Quality of life}},
  language     = {{eng}},
  pages        = {{3477--3485}},
  publisher    = {{Dove Medical Press Ltd.}},
  series       = {{International Journal of COPD}},
  title        = {{Lung Function and Health Status in Individuals with Severe Alpha-1-Antitrypsin Deficiency at the Age of 42}},
  url          = {{http://dx.doi.org/10.2147/COPD.S335683}},
  doi          = {{10.2147/COPD.S335683}},
  volume       = {{16}},
  year         = {{2021}},
}