{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T09:12:55Z","timestamp":1785143575335,"version":"3.55.0"},"reference-count":26,"publisher":"European Respiratory Society (ERS)","issue":"4","license":[{"start":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T00:00:00Z","timestamp":1771545600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["19AC5001"],"award-info":[{"award-number":["19AC5001"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["JPMH20AC5001"],"award-info":[{"award-number":["JPMH20AC5001"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["ERJ Open Res"],"accepted":{"date-parts":[[2026,1,27]]},"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD) with poor prognosis. Radiological pleuroparenchymal fibroelastosis (PPFE)-like findings, characterised by upper-lobe subpleural fibrosis, have been associated with worse outcome in IPF. While short leukocyte telomere length (LTL) is a recognised prognostic factor, its relationship with PPFE-like findings remains unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a secondary analysis of an ongoing ILD cohort. IPF patients who underwent LTL measurement by quantitative PCR were classified into those with PPFE-like findings on high-resolution computed tomography (IPF\/PPFE group) and those without such findings (IPF\/usual interstitial pneumonia (UIP) group). Clinical characteristics, pulmonary function, telomere length and outcomes were compared. Age-adjusted LTL was evaluated using healthy controls. Prognostic factors were analysed using Cox regression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among 179 IPF patients, 29 (16%) were assigned to the IPF\/PPFE group. Compared to the IPF\/UIP group (n=150), the IPF\/PPFE group had lower body mass index and forced vital capacity, and significantly shorter LTL (p=0.002), with more patients below the 10th percentile of healthy controls (37.9%\n                      <jats:italic>versus<\/jats:italic>\n                      12.0%). The IPF\/PPFE group showed greater respiratory functional decline and higher mortality (65.5%\n                      <jats:italic>versus<\/jats:italic>\n                      25.3%, p&lt;0.001). In survival analysis, both PPFE-like findings and shortened LTL predicted worse outcomes; however, only PPFE-like findings remained independently associated with mortality in multivariate analysis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>IPF patients with PPFE-like findings constitute a distinct high-risk phenotype with shorter telomeres, accelerated progression, and poor prognosis. These findings highlight the clinical importance of recognising PPFE-like changes and telomere biology in IPF for risk stratification and emphasise the need for close monitoring and early intervention.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1183\/23120541.01619-2025","type":"journal-article","created":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T12:00:16Z","timestamp":1771588816000},"page":"01619-2025","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Pleuroparenchymal fibroelastosis-like findings identify an idiopathic pulmonary fibrosis subgroup with shortened telomeres and poor 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