Abstract
BACKGROUND: Severe alpha 1-antitrypsin (AAT) deficiency (PiZZ) is associated with an increased risk of lung emphysema, especially in smokers. The aim of this study was to identify risk factors other than smoking for declining lung function. METHODS: Lung function was studied in 225 self-reported never-smoking PiZZ individuals included in the Swedish AAT deficiency register. RESULTS: Lung function was poorer in men than in women (mean (SD) forced expiratory volume in one second (FEV1) 80 (30) versus 88 (17)% predicted) despite the fact that the men were younger (mean (SD) age 45 (18) versus 51 (17) years), and poorer in those aged 50 or older than in those aged under 50 (mean (SD) FEV1 70 (30) versus 98 (16)% predicted). Self-reported occupational exposure to gas, fumes, or dust occurred more frequently in men than in women. In those aged 50 or older lung function was lower in individuals exposed to airway irritants than those who were not exposed (mean (SD) FEV1 63 (29) versus 76 (31)% predicted). Male sex, increasing age, and previous symptoms of wheezing were independent risk factors for lung function impairment, and male sex, wheeziness, and occupational exposure to airway irritants were independent risk factors in the subjects aged 50 years or more. CONCLUSIONS: In non-smoking PiZZ individuals lung function declines with increasing age, especially after 50. Men are at greater risk of lung function deterioration than women. Asthmatic symptoms and occupational exposure to airway irritants appear to constitute additional risk factors.
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- BERGLUND E., BIRATH G., BJURE J., GRIMBY G., KJELLMER I., SANDQVIST L., SODERHOLM B. Spirometric studies in normal subjects. I. Forced expirograms in subjects between 7 and 70 years of age. Acta Med Scand. 1963 Feb;173:185–192. [PubMed] [Google Scholar]
- Black L. F., Kueppers F. alpha1-Antitrypsin deficiency in nonsmokers. Am Rev Respir Dis. 1978 Mar;117(3):421–428. doi: 10.1164/arrd.1978.117.3.421. [DOI] [PubMed] [Google Scholar]
- Brantly M. L., Paul L. D., Miller B. H., Falk R. T., Wu M., Crystal R. G. Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis. 1988 Aug;138(2):327–336. doi: 10.1164/ajrccm/138.2.327. [DOI] [PubMed] [Google Scholar]
- Ferris B. G. Epidemiology Standardization Project (American Thoracic Society). Am Rev Respir Dis. 1978 Dec;118(6 Pt 2):1–120. [PubMed] [Google Scholar]
- Horne S. L., Tennent R. K., Cockcroft D. W., Cotton D. J., Dosman J. A. Pulmonary function in Pi M and MZ grainworkers. Chest. 1986 Jun;89(6):795–799. doi: 10.1378/chest.89.6.795. [DOI] [PubMed] [Google Scholar]
- Larsson C. Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z. Acta Med Scand. 1978;204(5):345–351. doi: 10.1111/j.0954-6820.1978.tb08452.x. [DOI] [PubMed] [Google Scholar]
- Laurell C. B. Electroimmuno assay. Scand J Clin Lab Invest Suppl. 1972;124:21–37. doi: 10.3109/00365517209102748. [DOI] [PubMed] [Google Scholar]
- Silverman E. K., Pierce J. A., Province M. A., Rao D. C., Campbell E. J. Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates. Ann Intern Med. 1989 Dec 15;111(12):982–991. doi: 10.7326/0003-4819-111-12-982. [DOI] [PubMed] [Google Scholar]
- Silverman E. K., Province M. A., Rao D. C., Pierce J. A., Campbell E. J. A family study of the variability of pulmonary function in alpha 1-antitrypsin deficiency. Quantitative phenotypes. Am Rev Respir Dis. 1990 Nov;142(5):1015–1021. doi: 10.1164/ajrccm/142.5.1015. [DOI] [PubMed] [Google Scholar]
- Tobin M. J., Cook P. J., Hutchison D. C. Alpha 1 antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association. Br J Dis Chest. 1983 Jan;77(1):14–27. doi: 10.1016/0007-0971(83)90002-5. [DOI] [PubMed] [Google Scholar]
- Wu M. C., Eriksson S. Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ. J Clin Epidemiol. 1988;41(12):1157–1165. doi: 10.1016/0895-4356(88)90019-4. [DOI] [PubMed] [Google Scholar]