{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T13:26:27Z","timestamp":1791897987014,"version":"4.3.4"},"reference-count":101,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2024,6,24]],"date-time":"2024-06-24T00:00:00Z","timestamp":1719187200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Pleuroparenchymal fibroelastosis (PPFE) is characterized by fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes. As PPFE appears to occur in patients with heterogeneous etiologies, the disease course is thus also heterogenous, with some patients showing rapid progression while others have slow progression. Therefore, it is very difficult to predict prognosis with PPFE. Needless to say, this problematic matter has influenced the treatment strategy of PPFE patients. In fact, until now no evidence has been shown for use in creating an appropriate management algorithm for PPFE. We speculate that \u201cuncoordinated breathing\u201d is the most important reason for dyspnea in PPFE patients. Because monitoring of physique and not just pulmonary function and radiological evaluation is also very important, particularly in PPFE patients, this review focused on the characteristics of PPFE through an overview of previous studies in this field, and we proposed an algorithm as precision medicine based on the current evidence. Multiple views by the pulmonologist are needed to standardize a clinical algorithm that is necessary to correctly assess PPFE patients under the premise of maintenance of physique by providing appropriate nutritional care and pulmonary rehabilitation.<\/jats:p>","DOI":"10.3390\/jcm13133675","type":"journal-article","created":{"date-parts":[[2024,6,24]],"date-time":"2024-06-24T06:59:58Z","timestamp":1719212398000},"page":"3675","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE)"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2480-6087","authenticated-orcid":false,"given":"Hideaki","family":"Yamakawa","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine, Saitama Red Cross Hospital, Saitama 330-8553, Japan"},{"name":"Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo 105-8461, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsuneyuki","family":"Oda","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keishi","family":"Sugino","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Tsuboi Hospital, Fukushima 963-0197, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9390-5057","authenticated-orcid":false,"given":"Takashi","family":"Hirama","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai 980-8575, Japan"},{"name":"Division of Organ Transplantation, Tohoku University, Sendai 980-8574, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1228-1490","authenticated-orcid":false,"given":"Masamichi","family":"Komatsu","sequence":"additional","affiliation":[{"name":"First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto 390-8621, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1321-3497","authenticated-orcid":false,"given":"Takuma","family":"Katano","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergology, Aichi Medical University, Nagakute 480-1195, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9173-738X","authenticated-orcid":false,"given":"Taiki","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Department of Radiology, The Jikei University School of Medicine, Tokyo 105-8461, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamiko","family":"Takemura","sequence":"additional","affiliation":[{"name":"Department of Pathology, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiaki","family":"Kubota","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Nippon Medical School, Tokyo 113-0022, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3335-5409","authenticated-orcid":false,"given":"Tomoo","family":"Kishaba","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Okinawa Chubu Hospital, Okinawa 904-2293, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhiro","family":"Norisue","sequence":"additional","affiliation":[{"name":"Department of Emergency and Critical Care Medicine, Tokyo Bay Urayasu Ichikawa Medical Center, Chiba 279-0001, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Araya","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo 105-8461, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Ogura","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,24]]},"reference":[{"key":"ref_1","first-page":"693","article-title":"Idiopathic pulmonary upper lobe fibrosis (IPUF)","volume":"11","author":"Amitani","year":"1992","journal-title":"Kokyu"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Tetikkurt, C., Ozturk, B.C., and Gungordu, N. 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