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Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis

The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study inves...

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Autores principales: Zaizen, Yoshiaki, Tachibana, Yuri, Kashima, Yukio, Bychkov, Andrey, Tabata, Kazuhiro, Otani, Kyoko, Kinoshita, Yoshiaki, Yamano, Yasuhiko, Kataoka, Kensuke, Ichikado, Kazuya, Okamoto, Masaki, Kishaba, Tomoo, Mito, Remi, Nishimura, Koichi, Yamasue, Mari, Nabeshima, Kazuki, Watanabe, Kentaro, Kondoh, Yasuhiro, Fukuoka, Junya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7956653/
https://www.ncbi.nlm.nih.gov/pubmed/33668178
http://dx.doi.org/10.3390/jcm10050895
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author Zaizen, Yoshiaki
Tachibana, Yuri
Kashima, Yukio
Bychkov, Andrey
Tabata, Kazuhiro
Otani, Kyoko
Kinoshita, Yoshiaki
Yamano, Yasuhiko
Kataoka, Kensuke
Ichikado, Kazuya
Okamoto, Masaki
Kishaba, Tomoo
Mito, Remi
Nishimura, Koichi
Yamasue, Mari
Nabeshima, Kazuki
Watanabe, Kentaro
Kondoh, Yasuhiro
Fukuoka, Junya
author_facet Zaizen, Yoshiaki
Tachibana, Yuri
Kashima, Yukio
Bychkov, Andrey
Tabata, Kazuhiro
Otani, Kyoko
Kinoshita, Yoshiaki
Yamano, Yasuhiko
Kataoka, Kensuke
Ichikado, Kazuya
Okamoto, Masaki
Kishaba, Tomoo
Mito, Remi
Nishimura, Koichi
Yamasue, Mari
Nabeshima, Kazuki
Watanabe, Kentaro
Kondoh, Yasuhiro
Fukuoka, Junya
author_sort Zaizen, Yoshiaki
collection PubMed
description The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study investigated the percentage of AED and the features of the denudated areas in 26 PPFE cases, 30 idiopathic pulmonary fibrosis (IPF) cases, and 29 controls. PPFE patients had lower forced vital capacities and higher residual volume/total lung capacities in pulmonary function tests compared to IPF and control patients. Histopathologically, subpleural fibroelastosis was observed in PPFE, and AED was observed in 12.01% of cases in the subpleural or interlobular septa regardless of fibroelastosis. The percentage of AED in the PPFE group was significantly higher than that in the IPF group (6.84%; p = 0.03) and the normal group (1.19%; p < 0.001). In the IPF group, the percentage of AED and the presence of PPFE-like lesions in the upper lobes were examined radiologically, but no correlation was found. We showed that AED frequently occurred in PPFE. AED was less frequent in IPF, which, in combination with imaging data, suggests that PPFE may have a different pathogenesis from IPF.
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spelling pubmed-79566532021-03-16 Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis Zaizen, Yoshiaki Tachibana, Yuri Kashima, Yukio Bychkov, Andrey Tabata, Kazuhiro Otani, Kyoko Kinoshita, Yoshiaki Yamano, Yasuhiko Kataoka, Kensuke Ichikado, Kazuya Okamoto, Masaki Kishaba, Tomoo Mito, Remi Nishimura, Koichi Yamasue, Mari Nabeshima, Kazuki Watanabe, Kentaro Kondoh, Yasuhiro Fukuoka, Junya J Clin Med Article The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study investigated the percentage of AED and the features of the denudated areas in 26 PPFE cases, 30 idiopathic pulmonary fibrosis (IPF) cases, and 29 controls. PPFE patients had lower forced vital capacities and higher residual volume/total lung capacities in pulmonary function tests compared to IPF and control patients. Histopathologically, subpleural fibroelastosis was observed in PPFE, and AED was observed in 12.01% of cases in the subpleural or interlobular septa regardless of fibroelastosis. The percentage of AED in the PPFE group was significantly higher than that in the IPF group (6.84%; p = 0.03) and the normal group (1.19%; p < 0.001). In the IPF group, the percentage of AED and the presence of PPFE-like lesions in the upper lobes were examined radiologically, but no correlation was found. We showed that AED frequently occurred in PPFE. AED was less frequent in IPF, which, in combination with imaging data, suggests that PPFE may have a different pathogenesis from IPF. MDPI 2021-02-24 /pmc/articles/PMC7956653/ /pubmed/33668178 http://dx.doi.org/10.3390/jcm10050895 Text en © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Zaizen, Yoshiaki
Tachibana, Yuri
Kashima, Yukio
Bychkov, Andrey
Tabata, Kazuhiro
Otani, Kyoko
Kinoshita, Yoshiaki
Yamano, Yasuhiko
Kataoka, Kensuke
Ichikado, Kazuya
Okamoto, Masaki
Kishaba, Tomoo
Mito, Remi
Nishimura, Koichi
Yamasue, Mari
Nabeshima, Kazuki
Watanabe, Kentaro
Kondoh, Yasuhiro
Fukuoka, Junya
Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
title Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
title_full Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
title_fullStr Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
title_full_unstemmed Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
title_short Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
title_sort alveolar epithelial denudation is a major factor in the pathogenesis of pleuroparenchymal fibroelastosis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7956653/
https://www.ncbi.nlm.nih.gov/pubmed/33668178
http://dx.doi.org/10.3390/jcm10050895
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