Progress of emphysema in severe α1-Antitrypsin Deficiency as Assessed by Annual CT

Purpose: To assess serial CT as a measure of the progress of emphysema in patients with severe α1,-antitrpsin deficiency (phenotype PiZ). Material and Methods: In a randomized placebo-controlled study of α1,-antitrypsin augmentation therapy, 22 patients with moderate emphysema were followed for 2–4 years with an annual lung CT. The images were analysed by means of semiautomatic lung detection, and the degree of emphysema was quantitated by the density-mask and the percentile methods. The influence of lung volume was standardised by a regression model. Results: A highly significant decline in Hounsfield units (HU) was found in low-density areas, corresponding to a mean (SE) annual loss of lung tissue of 2.1 (0.4) g/l lung volume. Analysis of a single slice at 5 cm below the level of the carina gave comparable results: 2.4 (0.4) g/l. Conclusion: Serial CT is a sensitive measure of the progress of emphysema in patients with severe α1-antitrypsin deficiency.

[1]  J. Roca,et al.  Standardization of the measurement of transfer factor (diffusing capacity). Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. , 1993, The European respiratory journal. Supplement.

[2]  Richard H. Jones.  Analysis of repeated measures , 1992 .

[3]  A. Miller,et al.  Standardized lung function testing. , 1984, Bulletin europeen de physiopathologie respiratoire.

[4]  H Adams,et al.  An appraisal of CT pulmonary density mapping in normal subjects. , 1991, Clinical radiology.

[5]  B. Burrows.  A clinical trial of efficacy of antiproteolytic therapy: can it be done? , 2015, The American review of respiratory disease.

[6]  L Kreel,et al.  Pulmonary tissue attenuation with computed tomography: comparison of inspiration and expiration scans. , 1979, Journal of computer assisted tomography.

[7]  J. Best,et al.  CT measurements of lung density in life can quantitate distal airspace enlargement--an essential defining feature of human emphysema. , 1988, The American review of respiratory disease.

[8]  Fagerhol Mk,et al.  The Pi polymorphism: genetic, biochemical, and clinical aspects of human alpha 1-antitrypsin. , 1981 .

[9]  J. Roca,et al.  Standardization of the measurement of transfer factor (diffusing capacity) , 1993, European Respiratory Journal.

[10]  F D Thomas,et al.  Density patterns in the normal lung as determined by computed tomography. , 1980, Radiology.

[11]  W A Kalender,et al.  Measurement of pulmonary parenchymal attenuation: use of spirometric gating with quantitative CT. , 1990, Radiology.

[12]  J C Yernault,et al.  Can computed tomography quantify pulmonary emphysema? , 1995, The European respiratory journal.

[13]  J E Cotes,et al.  Lung volumes and forced ventilatory flows. Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. , 1993, The European respiratory journal. Supplement.

[14]  J E Cotes,et al.  Lung volumes and forced ventilatory flows , 1993, European Respiratory Journal.

[15]  B Jonson,et al.  Measurement of pulmonary density by means of X-ray computerized tomography. Relation to pulmonary mechanics in normal subjects. , 1986, Chest.

[16]  A Heuck,et al.  Reproducibility of quantitative, spirometrically controlled CT. , 1995, Radiology.

[17]  N J Morrison,et al.  Quantitation of emphysema by computed tomography using a "density mask" program and correlation with pulmonary function tests. , 1990, Chest.

[18]  N. Müller,et al.  "Density mask". An objective method to quantitate emphysema using computed tomography. , 1988, Chest.

[19]  Flenley Dc.  Diagnosis and follow-up of emphysema. , 1990 .

[20]  W M Thurlbeck,et al.  Emphysema: definition, imaging, and quantification. , 1994, AJR. American journal of roentgenology.