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Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
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Platythorax progresses with lung involvement in pleuroparenchymal fibroelastosis
Takato Ikeda1, Yoshiaki Kinoshita1, Takuto Miyamura1
1Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan.
Respiratory Investigation
|November 4, 2021
Summary
Pleuroparenchymal fibroelastosis (PPFE) progression is linked to worsening platythorax. This study found a causal relationship between increasing lung fibrosis and chest flattening in PPFE patients.
Area of Science:
- Pulmonary Medicine
- Radiology
- Thoracic Surgery
Background:
- Pleuroparenchymal fibroelastosis (PPFE) is characterized by upper lobe lung disease and a flattened chest (platythorax).
- Platythorax is recognized as a marker of disease progression in PPFE.
- The precise temporal link between platythorax progression and lung involvement extent in PPFE remains under-investigated.
Purpose of the Study:
- To investigate the relationship between the progression of platythorax and the extent of lung involvement in patients with PPFE.
- To explore the temporal dynamics of fibrosis and chest morphology changes in PPFE.
Main Methods:
- Retrospective analysis of 29 patients diagnosed with PPFE.
- Estimation of lung fibrosis using a computed tomography (CT) fibrosis score.
- Measurement of thoracic cage dimensions using the flat chest index (anteroposterior to transverse diameter ratio).
- Assessment of serial changes in both the fibrosis score and flat chest index.
Main Results:
- A weak inverse correlation was observed between the fibrosis score and forced vital capacity %predicted at diagnosis (r = -0.40, p = 0.038).
- A moderate inverse correlation was found between the annual changes in the flat chest index and fibrosis score (r = -0.663, p = 0.0037).
Conclusions:
- The study suggests a causal relationship between the progression of lung fibroelastosis and the development of platythorax in PPFE patients.
- These findings highlight the interconnectedness of lung parenchymal changes and thoracic cage deformities in PPFE progression.
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