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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...
Autores principales: | , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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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. |
format | Online Article Text |
id | pubmed-7956653 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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