pleuroparenchymal fibroelastosis

Pathology

• pleural thickening by dense fibroelastotic tissue
• relatively confined to the pleura
• extension into the lung parenchyma along lobular septa
• deformation of normal pulmonary architecture in the subpleural region
• a few active fibroblastic foci in areas of fibrosis extending into the subjacent lung

Clinical manifestations

• progressive dyspnea over months (case report)

Laboratory

• apical wedge resection containing nodule (case report)
• elastic-tissue stain of surgical specimen

Radiology

• chest X-ray
  • pleural thickening
  • reticular linear peripheral opacities predominantly involving upper lung zones (case report)
• computed tomography (CT) of the chest (case report)
  • bilateral peripheral nodules associated with nodular pleural thickening extending into the fissures
  • linear bands in the parenchyma were adjacent to many peripheral nodules
  • no abnormalities were seen in lower lung
  • no hilar lymphadenopathy or mediastinal lymphadenopathy

Management

• observation
  • dyspnea may not worsen & patient may be able to perform his/her usual exercise routine (case report)
• lung transplantation ?
• minimize to iatrogenic stress (case report)

More general terms

• interstitial lung disease
• fibroelastosis