Abstract

Computed tomography (CT) has shown variability in the lung disease that occurs with alpha-1-antitrypsin deficiency (AATD). Basal emphysema has been the predominant recognized classical phenotype. CT has demonstrated that the pattern of emphysema is more varied and often involves the upper areas of the lung, thus a mixed phenotype is more common in AATD. Bronchiectasis has also been identified by CT in a proportion of AATD subjects suggesting that this is unlikely to be a coincidental finding. CT lung densitometry is also a useful parameter for a quantitative assessment of emphysema and is being used in clinical trials to measure responses to therapy.

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