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Published ahead of print on August 11, 2004, doi:10.1164/rccm.200406-761OC
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American Journal of Respiratory and Critical Care Medicine Vol 170. pp. 1172-1178, (2004)
© 2004 American Thoracic Society
doi: 10.1164/rccm.200406-761OC

Original Article

Pattern of Emphysema Distribution in {alpha}1-Antitrypsin Deficiency Influences Lung Function Impairment

David G. Parr, Berend C. Stoel, Jan Stolk and Robert A. Stockley

Lung Investigation Unit, Nuffield House, Queen Elizabeth Hospital, Birmingham, United Kingdom; Department of Radiology, Division of Image Processing; and Department of Pulmonology, Leiden University Medical Centre, Leiden, The Netherlands

Correspondence and requests for reprints should be addressed to Robert A. Stockley, D.Sc., Lung Investigation Unit, First Floor, Nuffield House, Queen Elizabeth Hospital, Birmingham, UK B15 2TH. E-mail: r.a.stockley{at}bham.ac.uk

FEV1 is fundamental to the diagnosis and staging of chronic obstructive pulmonary disease. In emphysema, airflow obstruction usually coexists with impairment of gas exchange, but discordance is not infrequent. We hypothesized that variations in the distribution of emphysema would be associated with functional differences and therefore account for discordant physiology. We used quantitative computed tomography to assess emphysema severity and distribution in 119 subjects with {alpha}1-antitrypsin deficiency (PiZ phenotype) and grouped them according to distribution pattern. In the 102 subjects with emphysema, 65 had a predominantly basal pattern ("basal"), but 37 (36%) had greater involvement of the upper regions ("apical"). Subjects from each group were matched for total volume of emphysema and age, and matched pairs analysis was used to relate emphysema distribution to clinical phenotype. Basal distribution was associated with greater impairment of FEV1 (mean difference, 9.9% predicted; 95% confidence interval, 3.8 to 16.0; p = 0.002) but less impairment of gas exchange (PaO2 mean difference, 0.5 kPa, 0.03 to 0.1; p = 0.016) and alveolar–arterial oxygen gradient (mean difference, 0.7 kPa; 0.2 to 1.2; p = 0.007) than the apical distribution. Emphysema distribution correlated with physiologic discordance (r = –0.409, p < 0.001). The use of single physiologic parameters as a surrogate measure of emphysema severity may introduce systematic bias in the staging of subjects with emphysema.

Key Words: {alpha}1-antitrypsin deficiency • computed tomography • lung densitometry




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