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Updated: Nov 26, 2025

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis
Yoshiaki Kinoshita1, Hiroshi Ishii2, Kazuki Nabeshima3
1Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan. y3kinoshitai@fukuoka-u.ac.jp y3kinoshita@gmail.com.
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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