Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare subtype of idiopathic interstitial pneumonia that consists of alveolar septal elastosis and intra-alveolar collagenosis, which is predominantly located in the upper lobes. The aim of this study was to examine the remodeling of the pulmona...
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| Veröffentlicht in: | Scientific reports 2020-01, Vol.10 (1), p.306-306, Article 306 |
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| description | Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare subtype of idiopathic interstitial pneumonia that consists of alveolar septal elastosis and intra-alveolar collagenosis, which is predominantly located in the upper lobes. The aim of this study was to examine the remodeling of the pulmonary arteries in patients with IPPFE. This study included 18 patients with IPPFE, 24 patients with idiopathic pulmonary fibrosis (IPF), and 5 patients without pulmonary disease as controls. We selected muscular pulmonary arteries and calculated the percentage of the thickness of each layer of the wall (intima, media, and adventitia) in relation to the external diameter. We also quantified the percentage of areas of elastic fiber in the media divided by the whole area of the media (medial elastic fiber score). The percentage of adventitial thickness in IPPFE was significantly higher than that in IPF and in control lungs. The percentage of medial thickness did not differ statistically between IPPFE and IPF. However, the medial elastic fiber score in IPPFE was also significantly larger than that in IPF and control lungs. These results suggest that collagenous thickening of the adventitia and medial elastosis are distinct histological features in the muscular pulmonary arteries of patients with IPPFE. |
| doi_str_mv | 10.1038/s41598-019-57248-3 |
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The aim of this study was to examine the remodeling of the pulmonary arteries in patients with IPPFE. This study included 18 patients with IPPFE, 24 patients with idiopathic pulmonary fibrosis (IPF), and 5 patients without pulmonary disease as controls. We selected muscular pulmonary arteries and calculated the percentage of the thickness of each layer of the wall (intima, media, and adventitia) in relation to the external diameter. We also quantified the percentage of areas of elastic fiber in the media divided by the whole area of the media (medial elastic fiber score). The percentage of adventitial thickness in IPPFE was significantly higher than that in IPF and in control lungs. The percentage of medial thickness did not differ statistically between IPPFE and IPF. However, the medial elastic fiber score in IPPFE was also significantly larger than that in IPF and control lungs. These results suggest that collagenous thickening of the adventitia and medial elastosis are distinct histological features in the muscular pulmonary arteries of patients with IPPFE.</description><identifier>ISSN: 2045-2322</identifier><identifier>EISSN: 2045-2322</identifier><identifier>DOI: 10.1038/s41598-019-57248-3</identifier><identifier>PMID: 31941942</identifier><language>eng</language><publisher>London: Nature Publishing Group UK</publisher><subject>692/420/256/2515 ; 692/699/1785/3193 ; Aged ; Alveoli ; Cardiac Catheterization ; Case-Control Studies ; Collagen ; Echocardiography ; Female ; Fibrosis ; Humanities and Social Sciences ; Humans ; Idiopathic Interstitial Pneumonias - pathology ; Idiopathic Pulmonary Fibrosis - pathology ; Lung diseases ; Male ; Middle Aged ; Mucin-1 - blood ; multidisciplinary ; Prognosis ; Pulmonary arteries ; Pulmonary artery ; Pulmonary Artery - pathology ; Pulmonary Artery - physiopathology ; Retrospective Studies ; Science ; Science (multidisciplinary) ; Thorax - diagnostic imaging ; Tomography, X-Ray Computed ; Ventricular Remodeling</subject><ispartof>Scientific reports, 2020-01, Vol.10 (1), p.306-306, Article 306</ispartof><rights>The Author(s) 2020</rights><rights>This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). 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Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Scientific reports</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kinoshita, Yoshiaki</au><au>Ishii, Hiroshi</au><au>Kushima, Hisako</au><au>Johkoh, Takeshi</au><au>Yabuuchi, Hidetake</au><au>Fujita, Masaki</au><au>Nabeshima, Kazuki</au><au>Watanabe, Kentaro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis</atitle><jtitle>Scientific reports</jtitle><stitle>Sci Rep</stitle><addtitle>Sci Rep</addtitle><date>2020-01-15</date><risdate>2020</risdate><volume>10</volume><issue>1</issue><spage>306</spage><epage>306</epage><pages>306-306</pages><artnum>306</artnum><issn>2045-2322</issn><eissn>2045-2322</eissn><abstract>Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare subtype of idiopathic interstitial pneumonia that consists of alveolar septal elastosis and intra-alveolar collagenosis, which is predominantly located in the upper lobes. The aim of this study was to examine the remodeling of the pulmonary arteries in patients with IPPFE. This study included 18 patients with IPPFE, 24 patients with idiopathic pulmonary fibrosis (IPF), and 5 patients without pulmonary disease as controls. We selected muscular pulmonary arteries and calculated the percentage of the thickness of each layer of the wall (intima, media, and adventitia) in relation to the external diameter. We also quantified the percentage of areas of elastic fiber in the media divided by the whole area of the media (medial elastic fiber score). The percentage of adventitial thickness in IPPFE was significantly higher than that in IPF and in control lungs. The percentage of medial thickness did not differ statistically between IPPFE and IPF. However, the medial elastic fiber score in IPPFE was also significantly larger than that in IPF and control lungs. These results suggest that collagenous thickening of the adventitia and medial elastosis are distinct histological features in the muscular pulmonary arteries of patients with IPPFE.</abstract><cop>London</cop><pub>Nature Publishing Group UK</pub><pmid>31941942</pmid><doi>10.1038/s41598-019-57248-3</doi><tpages>1</tpages><orcidid>https://orcid.org/0000-0002-2143-5922</orcidid><orcidid>https://orcid.org/0000-0002-8872-8957</orcidid><orcidid>https://orcid.org/0000-0001-5289-4905</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | 692/420/256/2515 692/699/1785/3193 Aged Alveoli Cardiac Catheterization Case-Control Studies Collagen Echocardiography Female Fibrosis Humanities and Social Sciences Humans Idiopathic Interstitial Pneumonias - pathology Idiopathic Pulmonary Fibrosis - pathology Lung diseases Male Middle Aged Mucin-1 - blood multidisciplinary Prognosis Pulmonary arteries Pulmonary artery Pulmonary Artery - pathology Pulmonary Artery - physiopathology Retrospective Studies Science Science (multidisciplinary) Thorax - diagnostic imaging Tomography, X-Ray Computed Ventricular Remodeling |
| title | Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis |
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