title: Emphysema Is Common in Lungs of Cystic Fibrosis Lung Transplantation Patients: A Histopathological and Computed Tomography Study.
abstract: BACKGROUND
  Lung disease in cystic fibrosis (CF) involves excessive inflammation, repetitive infections and development of bronchiectasis. Recently, literature on emphysema in CF has emerged, which might become an increasingly important disease component due to the increased life expectancy. The purpose of this study was to assess the presence and extent of emphysema in endstage CF lungs.
  
  
  METHODS
  In explanted lungs of 20 CF patients emphysema was semi-quantitatively assessed on histology specimens. Also, emphysema was automatically quantified on pre-transplantation computed tomography (CT) using the percentage of voxels below -950 Houndfield Units and was visually scored on CT. The relation between emphysema extent, pre-transplantation lung function and age was determined.
  
  
  RESULTS
  All CF patients showed emphysema on histological examination: 3/20 (15%) showed mild, 15/20 (75%) moderate and 2/20 (10%) severe emphysema, defined as 0-20% emphysema, 20-50% emphysema and >50% emphysema in residual lung tissue, respectively. Visually upper lobe bullous emphysema was identified in 13/20 and more diffuse non-bullous emphysema in 18/20. Histology showed a significant correlation to quantified CT emphysema (p = 0.03) and visual emphysema score (p = 0.001). CT and visual emphysema extent were positively correlated with age (p = 0.045 and p = 0.04, respectively).
  
  
  CONCLUSIONS
  In conclusion, this study both pathologically and radiologically confirms that emphysema is common in end-stage CF lungs, and is age related. Emphysema might become an increasingly important disease component in the aging CF population.
authors.affiliation: Department of Radiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Onno M
authors.initials: OM
authors.lastname: Mets
authors.affiliation: Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Suzan M
authors.initials: SM
authors.lastname: Roothaan
authors.affiliation: Department of Respiratory Medicine, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Inez
authors.initials: I
authors.lastname: Bronsveld
authors.affiliation: Department of Respiratory Medicine, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Bart
authors.initials: B
authors.lastname: Luijk
authors.affiliation: Department of Respiratory Medicine, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Ed A
authors.initials: EA
authors.lastname: van de Graaf
authors.affiliation: Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Aryan
authors.initials: A
authors.lastname: Vink
authors.affiliation: Department of Radiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Pim A
authors.initials: PA
authors.lastname: de Jong
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011656:Pulmonary Emphysema / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed