Publication:
The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis

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Kinoshita-36-291-303-2021.pdf(45.31 MB)
Date
2021
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Authors
Kinoshita, Yoshiaki ; Ishii, Hiroshi ; Nabeshima, Kazuki ; Watanabe, Kentaro
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DOI
https://doi.org/10.14670/HH-18-289
1699-5848
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info:eu-repo/semantics/article
Description
Abstract
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare subtype of idiopathic interstitial pneumonias that consists of elastofibrosis involving the lung parenchyma and pleural collagenous fibrosis predominantly located in the upper lobes. IPPFE has various distinct clinical and physiological characteristics, including platythorax and a marked decrease of forced vital capacity with an increased residual volume on a respiratory function test. The concept of IPPFE is now widely recognized and some diagnostic criteria have been proposed. In addition, the accumulation of cases has revealed the pathological features of IPPFE. However, little is known about the pathogenesis or the process of disease formation in IPPFE. This review article will provide a summary of the pathological features and previously reported hypotheses on disease formation in IPPFE, to discuss the potential etiologies and pathogenesis of IPPFE.
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Elastofibrosis , Elastic fiber , Etiology , Diagnostic criteria , Histopathology
Citation
Histology and Histopathology Vol. 36, nº3 (2021)
URI
http://hdl.handle.net/10201/127008
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Histology and histopathology Vol.36, nº3 (2021)
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