Dynamic Chest Radiography Visualizes Characteristic Respiratory Motion Impairment in Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report

ABSTRACT Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterized by upper lobe‐predominant pleural fibrosis and restrictive ventilatory impairment. However, methods for functional assessment of respiratory motion remain limited. We reported a 74‐year‐old woman who had severe restrictive impairment secondary to iPPFE, for which dynamic chest radiography (DCR) was used to evaluate respiratory mechanics. Quantitative DCR indices were compared with those of controls without pulmonary disease. DCR demonstrated markedly reduced diaphragmatic excursion, substantial decrease in respiratory lung area changes, and pronounced increase in low‐motion areas, indicating extensive regional motion restriction. These findings were consistent with impaired transmission of diaphragmatic motion due to pleural and subpleural fibrosis. This case illustrated that DCR can visualize and quantify characteristic respiratory motion abnormalities in iPPFE and may serve as a useful complementary modality to high‐resolution computed tomography and pulmonary function testing for functional assessment of iPPFE.

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Journal
Respirology Case Reports
Published
2026-03-31
DOI
https://doi.org/10.1002/rcr2.70573
Primary Topic
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
Type
article
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Dynamic Chest Radiography Visualizes Characteristic Respiratory Motion Impairment in Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report

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Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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Dynamic Chest Radiography Visualizes Characteristic Respiratory Motion Impairment in Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report

Kotaro Yamada, Naoki Koshimizu, Kensuke Kita, Ryuichi Nakamura, Norimichi Akiyama, Ryo Suzuki, Takumi Nagasaki, Kyohei Matsushita, Yuya Yamamoto, Shun Matsuura
article en

Abstract

ABSTRACT Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterized by upper lobe‐predominant pleural fibrosis and restrictive ventilatory impairment. However, methods for functional assessment of respiratory motion remain limited. We reported a 74‐year‐old woman who had severe restrictive impairment secondary to iPPFE, for which dynamic chest radiography (DCR) was used to evaluate respiratory mechanics. Quantitative DCR indices were compared with those of controls without pulmonary disease. DCR demonstrated markedly reduced diaphragmatic excursion, substantial decrease in respiratory lung area changes, and pronounced increase in low‐motion areas, indicating extensive regional motion restriction. These findings were consistent with impaired transmission of diaphragmatic motion due to pleural and subpleural fibrosis. This case illustrated that DCR can visualize and quantify characteristic respiratory motion abnormalities in iPPFE and may serve as a useful complementary modality to high‐resolution computed tomography and pulmonary function testing for functional assessment of iPPFE.

Respirology Case ReportsVol. 14(4)
Fujieda Municipal General Hospital (JP)
Good health and well-being
Openalex Percentile: Top 35%
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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