Science and Research

Similarities in the Computed Tomography Appearance in alpha1-Antitrypsin Deficiency and Smoking-Related Chronic Obstructive Pulmonary Disease in a Smoking Collective

BACKGROUND: Emphysematous destruction of lung parenchyma visible in computed tomography (CT) can be attributed to chronic obstructive pulmonary disease (COPD) or to alpha1-antitrypsin deficiency (AATD). OBJECTIVES: We evaluated if visual semiquantitative phenotyping of CT data helps identifying individuals with AATD in a group of smokers with severe emphysema and airflow limitation. METHOD: n = 14 patients with AATD and n = 15 with COPD and a minimum of 10 pack years underwent CT, clinical assessment, and full-body plethysmography. The extent and type of emphysema as well as large and small airway changes were rated semiquantitatively for each lobe using a standardized previously published scoring system. Lastly, a final diagnosis for each patient was proposed. RESULTS: AATD had a significantly lower mean emphysema score than COPD, with 8.9 +/- 3.4 versus 11.9 +/- 3.2 (p < 0.001), respectively. Within both groups, there was significantly more emphysema in the lower lobes (p < 0.05-0.001). The COPD group showed an upper- and middle-lobe predominance of emphysema distribution when compared to the AATD group (p < 0.001). Centrilobular (CLE) and panlobular (PLE) emphysema patterns showed a uniform distribution within both groups, with a CLE predominance in the upper lung and a PLE predominance in the lower lung regions. AATD and COPD both showed significantly more airway changes in lower lobes compared to upper lobes (p = 0.05-0.001), without significant differences between both groups. CONCLUSION: The typical emphysema distribution patterns seen on CT traditionally assigned to AATD and COPD were of little use in discriminating both entities. Also, airway changes could not contribute to a more precise differentiation. We conclude that a concise standardized phenotyping-driven approach to chest CT in emphysema is not sufficient to identify patients with AATD in a cohort of smokers with advanced emphysema.
  • Konietzke, P.
  • Jobst, B.
  • Wagner, W. L.
  • Jarosch, I.
  • Graber, R.
  • Kenn, K.
  • Kauczor, H. U.
  • Wielputz, M. O.

Keywords

  • Chronic obstructive pulmonary disease
  • Differentiation
  • Imaging
  • Smoker
  • alpha1-Antitrypsin deficiency
Publication details
DOI: 10.1159/000489177
Journal: Respiration; international review of thoracic diseases
Pages: 231-239 
Number: 3
Work Type: Original
Location: TLRC
Disease Area: COPD, PLI
Partner / Member: Thorax
Access-Number: 29940576
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