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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)
General
interstitial lung disease; diffuse parenchymal lung disease (DPLS, ILD)
fibroelastosis
References
- Shea BS, Sharma A, Mark EJ.
Case records of the Massachusetts General Hospital.
Case 14-2015. A 58-year-old woman with shortness of breath.
N Engl J Med. 2015 Apr 30;372(18):1749-58
PMID: 25923555
http://www.nejm.org/doi/full/10.1056/NEJMcpc1415200