Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics.

ABSTRACT: Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in 2013, its name refers to a combination of fibrosis involving the visceral pleura and fibroelastotic changes predominating in the subpleural lung parenchyma. Although a number of disease associations have been described, no single cause of PPFE has been unequivocally identified. A diagnosis of PPFE is most commonly achieved by identifying characteristic abnormalities on computed tomographic scans. The earliest changes are consistently located in the upper lobes close to the lung apices, the same locations where subsequent disease progression is also most conspicuous. When sufficiently severe, the

SUBMITTER: Chua F 

PROVIDER: S-EPMC6945468 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature