{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T12:30:29Z","timestamp":1789648229613,"version":"4.0.0"},"reference-count":34,"publisher":"European Respiratory Society (ERS)","issue":"3","license":[{"start":{"date-parts":[[2023,3,30]],"date-time":"2023-03-30T00:00:00Z","timestamp":1680134400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["22K08279"],"award-info":[{"award-number":["22K08279"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["ERJ Open Res"],"accepted":{"date-parts":[[2023,3,15]]},"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is characterised by upper lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma, with advanced cases often complicated by progressive weight loss. Therefore, we hypothesised that nutritional status is associated with mortality in IPPFE.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective study assesses nutritional status at the time of diagnosis and 1\u2005year after diagnosis in 131 patients with IPPFE. Malnutrition-related risk was evaluated using the Geriatric Nutritional Risk Index (GNRI).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 131 patients, 96 (73.8%) were at malnutrition-related risk at the time of diagnosis according to the GNRI. Of these, 21 patients (16.0%) were classified as at major malnutrition-related risk (GNRI &lt;82). Patients at major malnutrition-related risk were significantly older and had worse pulmonary function than patients at low (GNRI 92\u2013\u2009&lt;98) and moderate (GNRI 82\u2013\u2009&lt;92) malnutrition-related risk. GNRI scores decreased significantly from the time of diagnosis to 1\u2005year after diagnosis. Patients with a lower GNRI (&lt;91.8) had significantly shorter survival than patients with a median GNRI or higher (\u226591.8). Patients with declines in annual GNRI scores of \u22655 had significantly shorter survival than patients with declines in annual GNRI scores of &lt;5. In multivariate analysis, major malnutrition-related risk was significantly associated with increased mortality after adjustment for age, sex and forced vital capacity (hazard ratio 1.957). A composite scoring model including age, sex and major malnutrition-related risk was able to separate mortality risk in IPPFE.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Assessment of nutritional status by the GNRI provides useful information for managing patients with IPPFE by predicting mortality risk.<\/jats:p><\/jats:sec>","DOI":"10.1183\/23120541.00749-2022","type":"journal-article","created":{"date-parts":[[2023,3,30]],"date-time":"2023-03-30T15:21:51Z","timestamp":1680189711000},"page":"00749-2022","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":17,"title":["Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis"],"prefix":"10.1183","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6154-9791","authenticated-orcid":false,"given":"Yuzo","family":"Suzuki","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsuki","family":"Fukada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazutaka","family":"Mori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masato","family":"Kono","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotsugu","family":"Hasegawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dai","family":"Hashimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koshi","family":"Yokomura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiro","family":"Imokawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8075-0597","authenticated-orcid":false,"given":"Yusuke","family":"Inoue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideki","family":"Yasui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5439-1543","authenticated-orcid":false,"given":"Hironao","family":"Hozumi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0455-4070","authenticated-orcid":false,"given":"Masato","family":"Karayama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuki","family":"Furuhashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3187-4264","authenticated-orcid":false,"given":"Noriyuki","family":"Enomoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8809-1781","authenticated-orcid":false,"given":"Tomoyuki","family":"Fujisawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Inui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidenori","family":"Nakamura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takafumi","family":"Suda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2023,3,30]]},"reference":[{"key":"2024101711570756000_9.3.00749-2022.1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201308-1483ST"},{"key":"2024101711570756000_9.3.00749-2022.2","doi-asserted-by":"crossref","first-page":"1351","DOI":"10.1513\/AnnalsATS.201902-181CME","article-title":"Pleuroparenchymal fibroelastosis. 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