Progress of emphysema in severe alpha 1-antitrypsin deficiency as assessed by annual CT

Acta Radiol. 1997 Sep;38(5):826-32. doi: 10.1080/02841859709172418.


Purpose: To assess serial CT as a measure of the progress of emphysema in patients with severe alpha 1-antitrypsin deficiency (phenotype PiZ).

Material and methods: In a randomized placebo-controlled study of alpha 1-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 alpha 1-antitrypsin deficiency.

Publication types

  • Clinical Trial
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Disease
  • Disease Progression
  • Female
  • Humans
  • Lung / diagnostic imaging*
  • Male
  • Middle Aged
  • Phenotype
  • Pulmonary Emphysema / diagnostic imaging*
  • Pulmonary Emphysema / drug therapy
  • Pulmonary Emphysema / physiopathology
  • Regression Analysis
  • Respiratory Function Tests / methods
  • Respiratory Function Tests / statistics & numerical data
  • Time Factors
  • Tomography, X-Ray Computed / instrumentation
  • Tomography, X-Ray Computed / methods
  • Tomography, X-Ray Computed / statistics & numerical data
  • Trypsin Inhibitors / therapeutic use
  • alpha 1-Antitrypsin / therapeutic use
  • alpha 1-Antitrypsin Deficiency / diagnostic imaging*
  • alpha 1-Antitrypsin Deficiency / drug therapy
  • alpha 1-Antitrypsin Deficiency / physiopathology


  • Trypsin Inhibitors
  • alpha 1-Antitrypsin