{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T14:25:10Z","timestamp":1761920710770,"version":"3.37.3"},"reference-count":35,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,1,15]],"date-time":"2020-01-15T00:00:00Z","timestamp":1579046400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,1,15]],"date-time":"2020-01-15T00:00:00Z","timestamp":1579046400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"the Ministry of Health, Labor and Welfare of Japan awarded to the Study Group on Diffuse Pulmonary Disorders, Scientific Research\/Research on Intractable Diseases"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Sci Rep"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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.<\/jats:p>","DOI":"10.1038\/s41598-019-57248-3","type":"journal-article","created":{"date-parts":[[2020,1,15]],"date-time":"2020-01-15T11:03:00Z","timestamp":1579086180000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis"],"prefix":"10.1038","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8872-8957","authenticated-orcid":false,"given":"Yoshiaki","family":"Kinoshita","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2143-5922","authenticated-orcid":false,"given":"Hiroshi","family":"Ishii","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5289-4905","authenticated-orcid":false,"given":"Hisako","family":"Kushima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takeshi","family":"Johkoh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hidetake","family":"Yabuuchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaki","family":"Fujita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuki","family":"Nabeshima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kentaro","family":"Watanabe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,1,15]]},"reference":[{"key":"57248_CR1","doi-asserted-by":"publisher","first-page":"733","DOI":"10.1164\/rccm.201308-1483ST","volume":"188","author":"WD Travis","year":"2013","unstructured":"Travis, W. D. et al. An official American Thoracic Society\/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am. J. Respir. Crit. Care Med. 188, 733\u2013748 (2013).","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"57248_CR2","doi-asserted-by":"publisher","first-page":"2007","DOI":"10.1378\/chest.126.6.2007","volume":"126","author":"SK Frankel","year":"2004","unstructured":"Frankel, S. K., Cool, C. D., Lynch, D. A. & Brown, K. K. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 126, 2007\u201313 (2004).","journal-title":"Chest"},{"key":"57248_CR3","first-page":"693","volume":"11","author":"R Amitani","year":"1992","unstructured":"Amitani, R., Niimi, A. & Kuse, F. Idiopathic pulmonary upper lobe fibrosis. Kokyu 11, 693\u2013699 (1992).","journal-title":"Kokyu"},{"key":"57248_CR4","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1183\/09031936.00165111","volume":"40","author":"TL Reddy","year":"2012","unstructured":"Reddy, T. L. et al. Pleuroparenchymal fibroelastosis: A spectrum of histopathological and imaging phenotypes. Eur. Respir. J. 40, 377\u2013385 (2012).","journal-title":"Eur. Respir. J."},{"key":"57248_CR5","doi-asserted-by":"publisher","first-page":"1248","DOI":"10.1378\/chest.13-2866","volume":"146","author":"T Oda","year":"2014","unstructured":"Oda, T. et al. Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest 146, 1248\u20131255 (2014).","journal-title":"Chest"},{"key":"57248_CR6","first-page":"299","volume":"9","author":"K Watanabe","year":"2013","unstructured":"Watanabe, K. Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics. Curr. Respir. Med. Rev. 9, 299\u2013237 (2013).","journal-title":"Curr. Respir. Med. Rev."},{"key":"57248_CR7","doi-asserted-by":"publisher","first-page":"e9","DOI":"10.1016\/j.chest.2017.06.008","volume":"153","author":"S Harari","year":"2018","unstructured":"Harari, S., Elia, D. & Humbert, M. Pulmonary Hypertension in Parenchymal Lung Diseases: Any Future for New Therapies? Chest 153, e9\u2013e12 (2018).","journal-title":"Chest"},{"key":"57248_CR8","doi-asserted-by":"publisher","first-page":"2393","DOI":"10.1378\/chest.128.4.2393","volume":"128","author":"HF Nadrous","year":"2005","unstructured":"Nadrous, H. F. et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 128, 2393\u20139 (2005).","journal-title":"Chest"},{"key":"57248_CR9","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1164\/rccm.201401-0037OC","volume":"190","author":"J Hoffmann","year":"2014","unstructured":"Hoffmann, J. et al. Distinct differences in gene expression patterns in pulmonary arteries of patients with chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis with pulmonary hypertension. Am. J. Respir. Crit. Care Med. 190, 98\u2013111 (2014).","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"57248_CR10","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1165\/rcmb.2010-0365TR","volume":"45","author":"L Farkas","year":"2011","unstructured":"Farkas, L., Gauldie, J., Voelkel, N. F. & Kolb, M. Pulmonary hypertension and idiopathic pulmonary fibrosis: A tale of angiogenesis, apoptosis, and growth factors. Am. J. Respir. Cell Mol. Biol. 45, 1\u201315 (2011).","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"57248_CR11","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1007\/s00408-004-2542-z","volume":"183","author":"ER Parra","year":"2005","unstructured":"Parra, E. R. et al. Heterogeneous remodeling of lung vessels in idiopathic pulmonary fibrosis. Lung 183, 291\u2013300 (2005).","journal-title":"Lung"},{"key":"57248_CR12","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","volume":"37","author":"N Gali\u00e8","year":"2016","unstructured":"Gali\u00e8, N. et al. 2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur. Heart J. 37, 67\u2013119 (2016).","journal-title":"Eur. Heart J."},{"key":"57248_CR13","doi-asserted-by":"publisher","first-page":"639","DOI":"10.1016\/j.ccm.2013.08.009","volume":"34","author":"RM Tuder","year":"2013","unstructured":"Tuder, R. M., Stacher, E., Robinson, J., Kumar, R. & Graham, B. B. Pathology of pulmonary hypertension. Clin. Chest Med. 34, 639\u2013650 (2013).","journal-title":"Clin. Chest Med."},{"key":"57248_CR14","doi-asserted-by":"publisher","first-page":"533","DOI":"10.1161\/01.CIR.18.4.533","volume":"18","author":"D Heath","year":"1958","unstructured":"Heath, D. & Edwards, J. E. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 18, 533\u201347 (1958).","journal-title":"Circulation"},{"key":"57248_CR15","unstructured":"Katzenstein, A. L. A. Pulmonary hypertension and other vascular disorders. In Katzenstein and Askin\u2019s surgical pathology of non-neoplastic lung disease. (ed. Katzenstein, A. L. A.) 351\u2013383 (Elsevier, 2006)."},{"key":"57248_CR16","doi-asserted-by":"publisher","first-page":"643","DOI":"10.1007\/s00441-016-2539-y","volume":"367","author":"RM Tuder","year":"2017","unstructured":"Tuder, R. M. Pulmonary vascular remodeling in pulmonary hypertension. Cell Tissue Res. 367, 643\u2013649 (2017).","journal-title":"Cell Tissue Res."},{"key":"57248_CR17","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/1465-9921-15-34","volume":"15","author":"S Liu","year":"2014","unstructured":"Liu, S., Parameswaran, H., Young, S. M. & Varisco, B. M. JNK suppresses pulmonary fibroblast elastogenesis during alveolar development. Respir. Res. 15, 1\u201311 (2014).","journal-title":"Respir. Res."},{"key":"57248_CR18","doi-asserted-by":"publisher","first-page":"1608","DOI":"10.1016\/j.rmed.2013.08.008","volume":"107","author":"N Enomoto","year":"2013","unstructured":"Enomoto, N. et al. Amount of elastic fibers predicts prognosis of idiopathic pulmonary fibrosis. Respir. Med. 107, 1608\u201316 (2013).","journal-title":"Respir. Med."},{"key":"57248_CR19","doi-asserted-by":"publisher","first-page":"934","DOI":"10.1111\/his.13312","volume":"71","author":"Y Kinoshita","year":"2017","unstructured":"Kinoshita, Y. et al. Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole-slide image analysis and comparison with pleuroparenchymal fibroelastosis. Histopathology 71, 934\u2013942 (2017).","journal-title":"Histopathology"},{"key":"57248_CR20","doi-asserted-by":"publisher","first-page":"1370","DOI":"10.1183\/13993003.01537-2014","volume":"46","author":"G Raghu","year":"2015","unstructured":"Raghu, G. et al. Pulmonary hypertension in idiopathic pulmonary fibrosis with mild-to-moderaterestriction. Eur. Respir. J. 46, 1370\u20131377 (2015).","journal-title":"Eur. Respir. J."},{"key":"57248_CR21","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1183\/09031936.00107206","volume":"30","author":"AF Shorr","year":"2007","unstructured":"Shorr, A. F., Wainright, J. L., Cors, C. S., Lettieri, C. J. & Nathan, S. D. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur. Respir. J. 30, 715\u2013721 (2007).","journal-title":"Eur. Respir. J."},{"key":"57248_CR22","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1159\/000114246","volume":"76","author":"SD Nathan","year":"2008","unstructured":"Nathan, S. D. et al. Serial Development of Pulmonary Hypertension in Patients with Idiopathic Pulmonary Fibrosis. Respiration 76, 288\u2013294 (2008).","journal-title":"Respiration"},{"key":"57248_CR23","first-page":"352","volume":"23","author":"EB Boerner","year":"2017","unstructured":"Boerner, E. B., Costabel, U., Wessendorf, T. E., Theegarten, D. & Bonella, F. Idiopathic pleuroparenchymal fibroelastosis (PPFE) \u2013 A case study of a rare entity. Rev. Port. Pneumol. 23, 352\u2013355 (2017).","journal-title":"Rev. Port. Pneumol."},{"key":"57248_CR24","doi-asserted-by":"publisher","first-page":"1683","DOI":"10.1097\/PAS.0000000000000928","volume":"41","author":"R Khiroya","year":"2017","unstructured":"Khiroya, R. et al. Pleuroparenchymal fibroelastosis: A review of histopathologic features and the relationship between histologic parameters and survival. Am. J. Surg. Pathol. 41, 1683\u20131689 (2017).","journal-title":"Am. J. Surg. Pathol."},{"key":"57248_CR25","doi-asserted-by":"publisher","first-page":"536","DOI":"10.1111\/pin.12827","volume":"69","author":"Y Kinoshita","year":"2019","unstructured":"Kinoshita, Y., Watanabe, K., Ishii, H., Kushima, H. & Nabeshima, K. Lower\u2010lobe predominant pleuroparenchymal fibroelastosis. Pathol. Int. 69, 536\u2013540 (2019).","journal-title":"Pathol. Int."},{"key":"57248_CR26","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1164\/rccm.201201-0164OC","volume":"186","author":"E Stacher","year":"2012","unstructured":"Stacher, E. et al. Modern age pathology of pulmonary arterial hypertension. Am J Respir Crit Care Med. 186, 261\u2013272 (2012).","journal-title":"Am J Respir Crit Care Med."},{"key":"57248_CR27","doi-asserted-by":"publisher","first-page":"S25","DOI":"10.1016\/j.jacc.2004.02.033","volume":"43","author":"GG Pietra","year":"2004","unstructured":"Pietra, G. G. et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J. Am. Coll. Cardiol. 43, S25\u2013S32 (2004).","journal-title":"J. Am. Coll. Cardiol."},{"key":"57248_CR28","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.rmed.2017.11.003","volume":"133","author":"Y Enomoto","year":"2017","unstructured":"Enomoto, Y. et al. Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study. Respir. Med. 133, 1\u20135 (2017).","journal-title":"Respir. Med."},{"key":"57248_CR29","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1016\/j.resinv.2012.06.003","volume":"50","author":"K Watanabe","year":"2012","unstructured":"Watanabe, K. et al. Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Respir. Investig. 50, 88\u201397 (2012).","journal-title":"Respir. Investig."},{"key":"57248_CR30","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1016\/j.resinv.2019.02.007","volume":"57","author":"K Watanabe","year":"2019","unstructured":"Watanabe, K. et al. Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal. Respir. Investig. 57, 312\u2013320 (2019).","journal-title":"Respir. Investig."},{"key":"57248_CR31","doi-asserted-by":"publisher","first-page":"e44","DOI":"10.1164\/rccm.201807-1255ST","volume":"198","author":"G Raghu","year":"2018","unstructured":"Raghu, G. et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS\/ERS\/JRS\/ALAT Clinical Practice Guideline. Am. J. Respir. Crit. Care Med. 198, e44\u2013e68 (2018).","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"57248_CR32","doi-asserted-by":"publisher","first-page":"116","DOI":"10.1183\/09031936.00.15111600","volume":"15","author":"I Chazova","year":"2000","unstructured":"Chazova, I. et al. Venous and arterial changes in pulmonary veno-occlusive disease, mitral stenosis and fibrosing mediastinitis. Eur. Respir. J. 15, 116\u2013122 (2000).","journal-title":"Eur. Respir. J."},{"key":"57248_CR33","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1007\/s00428-018-2473-3","volume":"474","author":"Y Kinoshita","year":"2019","unstructured":"Kinoshita, Y. et al. Pleuroparenchymal fibroelastosis as a histological background of autoimmune diseases. Virchows Arch. 474, 97\u2013104 (2019).","journal-title":"Virchows Arch."},{"key":"57248_CR34","doi-asserted-by":"publisher","first-page":"875","DOI":"10.1016\/j.rmed.2012.02.015","volume":"106","author":"CU Andersen","year":"2012","unstructured":"Andersen, C. U. et al. Pulmonary hypertension in interstitial lung disease: Prevalence, prognosis and 6 min walk test. Respir. Med. 106, 875\u2013882 (2012).","journal-title":"Respir. Med."},{"key":"57248_CR35","doi-asserted-by":"publisher","first-page":"4015","DOI":"10.12659\/MSM.895033","volume":"21","author":"R Grabysa","year":"2015","unstructured":"Grabysa, R. & Wa\u0144kowicz, Z. Can Echocardiography, Especially Tricuspid Annular Plane Systolic Excursion Measurement, Predict Pulmonary Hypertension and Improve Prognosis in Patients on Long-Term Dialysis? Med. Sci. Monit. 21, 4015\u201322 (2015).","journal-title":"Med. Sci. Monit."}],"container-title":["Scientific Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41598-019-57248-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-019-57248-3","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-019-57248-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,6]],"date-time":"2022-12-06T05:27:58Z","timestamp":1670304478000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41598-019-57248-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,1,15]]},"references-count":35,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2020,12]]}},"alternative-id":["57248"],"URL":"https:\/\/doi.org\/10.1038\/s41598-019-57248-3","relation":{},"ISSN":["2045-2322"],"issn-type":[{"type":"electronic","value":"2045-2322"}],"subject":[],"published":{"date-parts":[[2020,1,15]]},"assertion":[{"value":"21 June 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 December 2019","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 January 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"306"}}