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Published ahead of print on August 11, 2004, doi:10.1164/rccm.200406-761OC

Am. J. Respir. Crit. Care Med., Volume 170, Number 11, December 2004, 1172-1178

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Submitted on June 16, 2004
Accepted on August 5, 2004

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

David G Parr1, Berend C Stoel2, Jan Stolk3, and Robert A Stockley1*

1 Lung Investigation Unit, Queen Elizabeth Hospital, Birmingham, Birmingham, West Midlands, United Kingdom, 2 Division of Image Processing, Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands, 3 Department of Pulmonology, Leiden University Medical Centre, Leiden, The Netherlands

* To whom correspondence should be addressed. E-mail: r.a.stockley{at}bham.ac.uk.

Forced Expiratory Volume in 1s. (FEV1) is fundamental to diagnosis/staging of COPD. In emphysema, airflow obstruction usually coexists with impairment of gas exchange, but discordance is not infrequent. We hypothesized that variations in emphysema distribution would be associated with functional differences, therefore accounting for discordant physiology. We used quantitative computed tomography to assess emphysema severity and distribution in 119 subjects with alpha 1-antitrypsin deficiency (PiZ) and grouped them according to distribution. In 102 subjects with emphysema, 65 had predominantly basal disease (~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). Use of single physiologic parameters as surrogate measures of severity may introduce systematic bias in staging of emphysema.
Key words: computed tomography; lung densitometry; alpha 1-antitrypsin deficiency




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