{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T23:08:29Z","timestamp":1775862509732,"version":"3.50.1"},"reference-count":23,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2023,5,4]],"date-time":"2023-05-04T00:00:00Z","timestamp":1683158400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background: Pleuroparenchymal Fibroelastosis (PPFE) is a rare disease that consists of elastofibrosis that involves the pleura and subpleural lung parenchyma; it is an unusual pulmonary disease with unique clinical, radiological and pathological characteristics. According to recent studies, PPFE may not be a definite disease but a form of chronic lung injury. The aim of this retrospective study is to determine the incidence and to evaluate the distribution, severity and progression of this radiological entity on high-resolution CT (HRCT) exams of the chest, performed in routine clinical practice. In total, 1514 HRCT exams performed in the period January 2016\u2013June 2018 were analyzed. For each exam, the presence of PPFE was evaluated and a quantitative score was assigned (from 0 to 7 points, based on the maximum depth of fibrotic involvement of the parenchyma). When available, two exams with a time interval of at least 6 months were compared for each patient in order to evaluate progression (defined as the increase in the disease score). Patients were divided into different groups according to exposure and their associated diseases. Statistical analysis was performed by using the Wilcoxon test and Kruskal\u2013Wallis test. Results: PPFE was detected in 174 out of 1514 patients (11.6%), with a mean score of 6.1 \u00b1 3.9 (range 1\u201314). In 106 out of 174 patients (60.9%), a previous CT scan was available and an evolution of PPFE was detected in 19 of these (11.5%). Among these 19 patients with worsening PPFE, 4 had isolated PPFE that was associated with chronic exposure or connective tissue disorders, and the other 15 had an associated lung disease and\/or a chronic exposure. In this group, it was found that the ventral segments of the upper lobes, fissures and apical segments of the lower lobes had a greater statistically significant involvement in the progression of the disease compared to the non-progressive group. In 16 of 174 patients (9.2%, 7 of which belonged to the radiological progression group) a biopsy through video-assisted thoracoscopic surgery or apicoectomy confirmed PPFE. Conclusion: PPFE-like lesions are not uncommon on HRCT exams in routine clinical practice, and are frequently found in patients with different forms of chronic lung injury. Further studies are necessary to explain why the disease progresses in some cases, while in most, it remains stationary over time.<\/jats:p>","DOI":"10.3390\/diagnostics13091627","type":"journal-article","created":{"date-parts":[[2023,5,5]],"date-time":"2023-05-05T01:38:21Z","timestamp":1683250701000},"page":"1627","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Pleuroparenchymal Fibroelastosis-like Lesions in Clinical Practice: A Rare Entity? Review of a Radiological Database"],"prefix":"10.3390","volume":"13","author":[{"given":"Francesco","family":"Gentili","sequence":"first","affiliation":[{"name":"Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vito","family":"Di Martino","sequence":"additional","affiliation":[{"name":"Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Forestieri","sequence":"additional","affiliation":[{"name":"Diagnostic Imaging Bambino Ges\u00f9 Hospital, 00165 Roma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Mazzei","sequence":"additional","affiliation":[{"name":"Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7875-3342","authenticated-orcid":false,"given":"Susanna","family":"Guerrini","sequence":"additional","affiliation":[{"name":"Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8351-3703","authenticated-orcid":false,"given":"Elena","family":"Bargagli","sequence":"additional","affiliation":[{"name":"Respiratory Diseases and Lung Transplant Unit, Department of Medical and Surgical Sciences and Neurosciences, University of Siena, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonietta Gerardina","family":"Sisinni","sequence":"additional","affiliation":[{"name":"Unit of Occupational Medicine, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Volterrani","sequence":"additional","affiliation":[{"name":"Department of Radiological Sciences, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Antonietta","family":"Mazzei","sequence":"additional","affiliation":[{"name":"Department of Radiological Sciences, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,5,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1164\/rccm.201308-1483ST","article-title":"An official American Thoracic Society\/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias","volume":"188","author":"Travis","year":"2013","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_2","first-page":"693","article-title":"Idiopathic Pulmonary Upper Lobe Fibrosis (IPUF)","volume":"11","author":"Amitani","year":"1992","journal-title":"Kokyu"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2007","DOI":"10.1378\/chest.126.6.2007","article-title":"Idiopathic pleuroparenchymal fibroelastosis: Description of a novel clinicopathologic entity","volume":"126","author":"Frankel","year":"2004","journal-title":"Chest"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"229","DOI":"10.2174\/1573398X0904140129125307","article-title":"Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics","volume":"9","author":"Watanabe","year":"2013","journal-title":"Curr. Respir. Med. Rev."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1183\/09031936.00165111","article-title":"Pleuroparenchymal fibroelastosis: A spectrum of histopathological and imaging phenotypes","volume":"40","author":"Reddy","year":"2012","journal-title":"Eur. Respir. J."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1378\/chest.13-2866","article-title":"Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis","volume":"146","author":"Oda","year":"2014","journal-title":"Chest"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1351","DOI":"10.1513\/AnnalsATS.201902-181CME","article-title":"Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics","volume":"16","author":"Chua","year":"2019","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1183\/09031936.00214714","article-title":"Pleuroparenchymal fibroelastosis from a consecutive database: A rare disease entity?","volume":"45","author":"Nakatani","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.humpath.2014.10.007","article-title":"Pleuroparenchymal fibroelastosis: A pattern of chronic lung injury","volume":"46","author":"Rosenbaum","year":"2015","journal-title":"Hum. Pathol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e16086","DOI":"10.1097\/MD.0000000000016086","article-title":"Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis: What should the rheumatologist do?","volume":"98","author":"Bargagli","year":"2019","journal-title":"Medicine"},{"key":"ref_11","first-page":"100843","article-title":"Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype?","volume":"27","author":"Perruzza","year":"2019","journal-title":"Respir. Med. Case Rep."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"6797826","DOI":"10.1155\/2017\/6797826","article-title":"Incidental and Underreported Pleural Plaques at Chest CT: Do Not Miss Them\u2014Asbestos Exposure Still Exists","volume":"2017","author":"Mazzei","year":"2017","journal-title":"BioMed Res. Int."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1007\/s11604-018-0805-5","article-title":"Pleuroparenchymal fibroelastosis-like lesions on chest computed tomography in routine clinical practice","volume":"37","author":"Sumikawa","year":"2019","journal-title":"Jpn. J. Radiol."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Enomoto, Y., Nakamura, Y., Colby, T.V., Johkoh, T., Sumikawa, H., Nishimoto, K., Yoshimura, K., Matsushima, S., Oyama, Y., and Hozumi, H. (2017). Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0180283"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1053\/j.sult.2018.10.019","article-title":"Occupational Lung Diseases: Underreported Diagnosis in Radiological Practice","volume":"40","author":"Mazzei","year":"2019","journal-title":"Semin. Ultrasound CT MRI"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1097\/00000478-200105000-00018","article-title":"Pulmonary apical cap: A distinctive but poorly recognized lesion in pulmonary surgical pathology","volume":"25","author":"Yousem","year":"2001","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.5858\/arpa.2015-0224-RA","article-title":"Pulmonary Apical Cap-What\u2019s Old Is New Again","volume":"139","author":"Lagstein","year":"2015","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"299","DOI":"10.2214\/ajr.137.2.299","article-title":"The apical cap","volume":"137","author":"McLoud","year":"1981","journal-title":"AJR Am. J. Roentgenol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1002\/path.1700450311","article-title":"Apical scars and their relationship to siliceous dust accumulation in non-silicotic lungs","volume":"45","author":"Davson","year":"1937","journal-title":"J. Pathol. Bacteriol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1002\/path.1700490304","article-title":"The early stages of apical scar development","volume":"49","author":"Davson","year":"1939","journal-title":"J. Pathol. Bacteriol."},{"key":"ref_21","first-page":"205","article-title":"The pulmonary apical cap","volume":"60","author":"Butler","year":"1970","journal-title":"Am. J. Pathol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"849","DOI":"10.5858\/arpa.2015-0166-RS","article-title":"Pleuroparenchymal Fibroelastosis of the Lung: A Review","volume":"140","author":"Cheng","year":"2016","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1159\/000494061","article-title":"Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis","volume":"97","author":"Kato","year":"2019","journal-title":"Respiration"}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/9\/1627\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:29:12Z","timestamp":1760124552000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/9\/1627"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,4]]},"references-count":23,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2023,5]]}},"alternative-id":["diagnostics13091627"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics13091627","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,4]]}}}