2024
DOI: 10.3390/jcm13133675
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Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE)

Hideaki Yamakawa,
Tsuneyuki Oda,
Keishi Sugino
et al.

Abstract: Pleuroparenchymal fibroelastosis (PPFE) is characterized by fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes. As PPFE appears to occur in patients with heterogeneous etiologies, the disease course is thus also heterogenous, with some patients showing rapid progression while others have slow progression. Therefore, it is very difficult to predict prognosis with PPFE. Needless to say, this problematic matter has influenced the treatment strategy of PPFE patients. In … Show more

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