{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T10:49:39Z","timestamp":1791110979203,"version":"4.1.0"},"reference-count":26,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2020,3,9]],"date-time":"2020-03-09T00:00:00Z","timestamp":1583712000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["NRF\u20102019R1A2C2008541"],"award-info":[{"award-number":["NRF\u20102019R1A2C2008541"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background and objective<\/jats:title><jats:p>PPFE is characterized by fibrosis in the pleura and subpleural lung parenchyma in the upper lobes, while other types of ILD, mainly UIP, can be observed in about half of the patients in their lower lobes. The aim of this study was to evaluate the clinical significance of the radiologically defined PPFE in patients with IPF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Clinical data and chest CT images were retrospectively analysed in 445 patients with IPF (biopsy\u2010proven cases, <jats:italic>n<\/jats:italic> = 165). The radiological criteria of PPFE were defined as follows: (i) bilateral subpleural dense fibrosis with or without pleural thickening in the upper lobes, (ii) evidence of disease progression and (iii) no clinical evidence of identifiable aetiologies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The median follow\u2010up period was 43.0 months. The mean age of the patients was 66.4\u2009years and 76.4% were male. PPFE was identified in 28 patients (6.3%). The PPFE group showed lower BMI and lung function (FVC and TLC) at baseline, more frequent pneumothorax and pneumomediastinum, higher decline rates in lung function and poorer prognosis during follow\u2010up than the no\u2010PPFE group. PPFE was an independent risk factor (HR = 2.953, 95% CI: 1.350\u20136.460, <jats:italic>P<\/jats:italic> = 0.007) for pneumothorax or pneumomediastinum, but not for mortality in patients with IPF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Among patients with IPF, the PPFE group, when compared to the no\u2010PPFE group, showed lower BMI and lung function and showed more frequent complications and poorer survival during follow\u2010up.<\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.13796","type":"journal-article","created":{"date-parts":[[2020,3,9]],"date-time":"2020-03-09T07:36:30Z","timestamp":1583739390000},"page":"1046-1052","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis"],"prefix":"10.1111","volume":"25","author":[{"given":"Song\u2010I.","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Pulmonary and Critical Care Medicine, Asan Medical Center University of Ulsan College of Medicine  Seoul Republic of Korea"},{"name":"Department of Pulmonary and Critical Care Medicine Chungnam National University Hospital, Chungnam National University School of Medicine  Daejeon Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eun Jin","family":"Chae","sequence":"additional","affiliation":[{"name":"Department of Radiology, Asan Medical Center University of Ulsan College of Medicine  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joon Seon","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Pathology, Asan Medical Center University of Ulsan College of Medicine  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0932-2826","authenticated-orcid":false,"given":"Jae Ha","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Department of Internal Medicine Inje University College of Medicine, Haeundae Paik Hospital  Busan Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5121-3522","authenticated-orcid":false,"given":"Jin Woo","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Pulmonary and Critical Care Medicine, Asan Medical Center University of Ulsan College of Medicine  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,3,9]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201308-1483ST"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2017.11.003"},{"key":"e_1_2_9_4_1","first-page":"299","article-title":"Pleuroparenchymal fibroelastosis: its clinical characteristics","volume":"9","author":"Watanabe K","year":"2013","journal-title":"Curr. 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