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Published ahead of print on September 13, 2007, doi:10.1164/rccm.200703-489OC

Am. J. Respir. Crit. Care Med., Volume 176, Number 12, December 2007, 1215-1221

A more recent version of this article appeared on December 15, 2007
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Submitted on March 26, 2007
Accepted on September 13, 2007

The Prevalence and Impact of Bronchiectasis in Alpha-1 Antitrypsin Deficiency

David G Parr1*, Peter G Guest2, John H Reynolds3, Lee J Dowson4, and Robert A Stockley5

1 Department of Respiratory Medicine, University Hospitals of Coventry and Warwickshire, Coventry, West Midlands, United Kingdom; Lung Investigation Unit, University Hospital, Birmingham, Birmingham, West Midlands, United Kingdom, 2 Department of Radiology, University Hospital, Birmingham, Birmingham, West Midlands, United Kingdom, 3 Department of Radiology, Birmingham Heartlands Hospital, Birmingham, West Midlands, United Kingdom, 4 Department of Respiratory Medicine, Royal Wolverhampton Hospitals, Wolverhampton, West Midlands, United Kingdom, 5 Lung Investigation Unit, University Hospital, Birmingham, Birmingham, West Midlands, United Kingdom

* To whom correspondence should be addressed. E-mail: david.parr{at}uhcw.nhs.uk.

Rationale: Alpha-1 antitrypsin (AAT) deficiency is associated with an increased risk of COPD, in particular emphysema, but airways disease is less well described. Methods: We characterized the CT phenotype of 74 subjects (PiZ) using visual scoring of airways disease and densitometric assessment of emphysema. CT measurements were related to physiology, health status, and emphysema severity, and the relative impact of airways disease and emphysema severity on health status and airflow obstruction was compared by stepwise regression. Measurements and Main Results: Bronchiectatic changes were seen in 70 subjects, and a subgroup with a bronchiectasis-predominant phenotype was identified. Clinically significant bronchiectasis (radiologic bronchiectasis in four or more bronchopulmonary segments together with symptoms of regular sputum production) occurred in 20 subjects (27%). Index cases had higher airways disease scores (p < 0.05), more severe emphysema (p < 0.001), and greater impairment of physiology (p < 0.001) and health status (p < 0.05) than non-index cases. Airways disease scores correlated with health status, and bronchial wall thickening correlated with FEV1. Emphysema severity had the strongest associations for health status (R = 0.505, p < 0.001) and FEV1 (R = 0.699, p < 0.001), but the addition of airways disease score improved the regression models (R = 0.596, p = 0.002 and R = 0.783, p < 0.001, respectively). Conclusions: Emphysema is the predominant component of COPD in AAT deficiency, but the prevalence and impact of airways disease are greater than currently recognized. Future therapeutic strategies in AAT deficiency should target this component of COPD.
Key words: Chronic obstructive pulmonary disease; emphysema, bronchiectasis, computed tomography, alpha 1-Antitryspin deficiency




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