title: Correlation between annual change in health status and computer tomography derived lung density in subjects with alpha1-antitrypsin deficiency.
abstract: BACKGROUND
  There is increasing recognition that questionnaires of health status and lung density measurements are more sensitive tools for assessing progression of emphysema than forced expiratory volume in 1 second (FEV(1)) and transfer coefficient (KCO). A study was undertaken to investigate prospectively the correlation between annual change in health status and computer tomography (CT) derived lung density in subjects with alpha(1)-antitrypsin deficiency.
  
  
  METHODS
  Twenty two patients of mean (SD) age 40.7 (9.2) years with ZZ type alpha(1)-antitrypsin deficiency were investigated at baseline and 30 months later by FEV(1) and KCO, St George Respiratory Questionnaire (SGRQ), and by a spiral CT scan of the chest. CT data of chest images were analysed using software designed for automated lung contour detection and lung density measurements. The density data were corrected for changes in inspiration levels.
  
  
  RESULTS
  Changes in lung density, expressed as 15th percentile point or relative area below -950 HU, correlated well with changes in health status (SGRQ total score): R = -0.56, p = 0.007 or R = 0.6, p = 0.003. Neither changes in health status nor changes in lung density correlated significantly with changes in FEV(1) or changes in KCO.
  
  
  CONCLUSIONS
  The SGRQ total score (which is a global measure in COPD) and lung density (a specific measure of emphysema) are sensitive to deterioration in patients with alpha(1)-antitrypsin deficiency. This finding may facilitate future studies with new drugs specific for emphysema, a frequently occurring component of COPD.
authors.affiliation: Department of Pulmonology, Leiden University Medical Center, Leiden, The Netherlands [corrected]. j.stolk.long@lumc.nl
authors.forename: J
authors.initials: J
authors.lastname: Stolk
authors.forename: W H
authors.initials: WH
authors.lastname: Ng
authors.forename: M E
authors.initials: ME
authors.lastname: Bakker
authors.forename: J H C
authors.initials: JH
authors.lastname: Reiber
authors.forename: K F
authors.initials: KF
authors.lastname: Rabe
authors.forename: H
authors.initials: H
authors.lastname: Putter
authors.forename: B C
authors.initials: BC
authors.lastname: Stoel
mesh_terms: D000328:Adult; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006304:Health Status; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D011446:Prospective Studies; D029424:Pulmonary Disease, Chronic Obstructive / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D011656:Pulmonary Emphysema / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D014057:Tomography, X-Ray Computed / Q000379:methods*; D019896:alpha 1-Antitrypsin Deficiency / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology