Regional lung densities in alpha-1 antitrypsin deficiency compared to predicted values.

ABSTRACT

BACKGROUND: We developed a method to calculate a standard score for lung tissue mass derived from CT scan images from a control group without respiratory disease. We applied the method to images from subjects with emphysema associated with alpha-1 antitrypsin deficiency (AATD) and used it to study regional patterns of differential tissue mass. METHODS: We explored different covariates in 76 controls. Standardization was applied to facilitate comparability between different CT scanners and a standard Z-score (Standard Mass Score, SMS) was developed, representing lung tissue loss compared to normal lung mass. This normative data was defined for the entire lungs and for delineated apical, central and basal regions. The agreement with DLCO%pred was explored in a data set of 180 patients with emphysema who participated in a trial of alpha-1-antitrypsin augmentation treatment (RAPID). RESULTS: Large differences between emphysematous and normal tissue of more than 10 standard deviations were found. There was reasonable agreement between SMS and DLCO%pred for the global densitometry (κ = 0.252, p < 0.001), varying from κ = 0.138 to κ = 0.219 and 0.264 (p < 0.001), in the apical, central and basal region, respectively. SMS and DLCO%pred correlated consistently across apical, central and basal regions. The SMS distribution over the different lung regions showed a distinct pattern suggesting that emphysema due to severe AATD develops from basal to central and ultimately apical regions. CONCLUSIONS: Standardization and normalization of lung densitometry is feasible and the adoption of the developed principles helps to characterize the distribution of emphysema, required for clinical decision making.