title: [Combined pulmonary fibrosis and emphysema: the natural history of the disease. The chronological evolution of clinical features, respiratory function and the CT scan].
abstract: BACKGROUND
  The syndrome of combined basal pulmonary fibrosis and apical emphysema (CPFE) is characterised by severe dyspnoea not fully explained by pulmonary function tests that show subnormal lung volumes and expiratory flows while CT imaging reveals varying degrees of emphysema and interstitial fibrosis.
  
  
  METHODS
  A man presented with chronic obstructive pulmonary disease associated with severe emphysema. Airflow obstruction was associated with early over-inflation. Five years later he developed interstitial fibrosis with CT appearances compatible with non-specific interstitial pneumonitis. Simultaneously, the decreased expiratory flows due to emphysema had become normal and the pulmonary distension had resolved.
  
  
  CONCLUSIONS
  When CPFE develops, the reduced expiratory flows and thoracic distension due to emphysema can resolve. These changes may be explained by the late development of interstitial changes, which balance the functional consequences of emphysema by an increase in elastic recoil and reduction in volume. This is the first case report that describes the evolutionary profile in a single patient. It reinforces the pathophysiological hypothesis that explains the normal pulmonary volumes and expiratory flows in CPFE.
authors.affiliation: Service des maladies respiratoires et réanimation respiratoire, CHU de Nancy-Brabois, 10, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France. rponcot-mongars@voila.fr
authors.forename: R
authors.initials: R
authors.lastname: Ponçot-Mongars
authors.forename: M
authors.initials: M
authors.lastname: Zysman
authors.forename: D
authors.initials: D
authors.lastname: Regent
authors.forename: E
authors.initials: E
authors.lastname: Gomez
authors.forename: A
authors.initials: A
authors.lastname: Chaouat
authors.forename: F
authors.initials: F
authors.lastname: Chabot
mesh_terms: D000368:Aged; D018450:Disease Progression; D004417:Dyspnea / Q000209:etiology; D004646:Emphysema / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000503:physiopathology; D000860:Hypoxia / Q000209:etiology; D008168:Lung / Q000473:pathology / Q000503:physiopathology; D008176:Lung Volume Measurements; D008297:Male; D009765:Obesity / Q000150:complications; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology; D011658:Pulmonary Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D012907:Smoking / Q000009:adverse effects; D014057:Tomography, X-Ray Computed