{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T20:44:35Z","timestamp":1778618675858,"version":"3.51.4"},"reference-count":34,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,6,12]],"date-time":"2024-06-12T00:00:00Z","timestamp":1718150400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,6,12]],"date-time":"2024-06-12T00:00:00Z","timestamp":1718150400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"The Research Foundation of North Finland","award":["No ID number"],"award-info":[{"award-number":["No ID number"]}]},{"name":"The Research Foundation of Pulmonary Diseases","award":["No ID number"],"award-info":[{"award-number":["No ID number"]}]},{"name":"The Jalmari and Rauha Ahokas Foundation","award":["No ID number"],"award-info":[{"award-number":["No ID number"]}]},{"name":"The Tampere Tuberculosis Foundation","award":["No ID number"],"award-info":[{"award-number":["No ID number"]}]},{"name":"A state subsidy from Oulu University Hospital","award":["No ID number"],"award-info":[{"award-number":["No ID number"]}]},{"DOI":"10.13039\/501100006196","name":"University of Oulu","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100006196","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Respir Res"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>In familial pulmonary fibrosis (FPF) at least two biological relatives are affected. Patients with FPF have diverse clinical features.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Research question<\/jats:title>\n                <jats:p>We aimed to characterize demographic and clinical features, re-evaluate high-resolution computed tomography (HRCT) scans and histopathology of surgical lung biopsies, assess survival and investigate the suitability of risk prediction models for FPF patients.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Study design<\/jats:title>\n                <jats:p>A retrospective cohort study.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>FPF data (<jats:italic>n<\/jats:italic> = 68) were collected from the medical records of Oulu University Hospital (OUH) and Oulaskangas District Hospital between 1 Jan 2000 and 11 Jan 2023. The inclusion criterion was pulmonary fibrosis (PF) (ICD 10-code J84.X) and at least one self-reported relative with PF. Clinical information was gathered from hospital medical records. HRCT scans and histology were re-evaluated.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Thirty-seven (54.4%) of the patients were men, and 31 (45.6%) were women. The mean ages of the women and men were 68.6 and 61.7 years, respectively (<jats:italic>p<\/jats:italic> = 0.003). Thirty-seven (54.4%) patients were nonsmokers. The most common radiological patterns were usual interstitial pneumonia (UIP) (51\/75.0%), unclassifiable (8\/11.8%) and nonspecific interstitial pneumonia (NSIP) (3\/4.4%). Pleuroparenchymal fibroelastosis (PPFE) was observed as a single or combined pattern in 13.2% of the patients. According to the 2022 guidelines for idiopathic pulmonary fibrosis (IPF), the patients were categorized as UIP (31\/45.6%), probable UIP (20\/29.4%), indeterminate for UIP (7\/10.3%) or alternative diagnosis (10\/14.7%). The histopathological patterns were UIP (7\/41.2%), probable UIP (1\/5.9%), indeterminate for UIP (8\/47.2%) and alternative diagnosis (1\/5.9%). Rare genetic variants were found in 9 patients; these included telomerase reverse transcriptase (<jats:italic>TERT<\/jats:italic>, <jats:italic>n<\/jats:italic> = 6), telomerase RNA component (<jats:italic>TERC<\/jats:italic>, <jats:italic>n<\/jats:italic> = 2) and regulator of telomere elongation helicase 1 (<jats:italic>RTEL1<\/jats:italic>, <jats:italic>n<\/jats:italic> = 1). Half of the patients died (<jats:italic>n<\/jats:italic> = 29) or underwent lung transplantation (<jats:italic>n<\/jats:italic> = 5), with a median survival of 39.9 months. The risk prediction models composite physiology index (CPI), hazard ratio (HR) 1.07 (95.0% CI 1.04\u20131.10), and gender-age-physiology index (GAP) stage I predicted survival statistically significantly (<jats:italic>p<\/jats:italic>&lt;0.001) compared to combined stages II and III.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>This study confirmed the results of earlier studies showing that FPF patients\u2019 radiological and histopathological patterns are diverse. Moreover, radiological and histological features revealed unusual patterns and their combinations.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12931-024-02864-5","type":"journal-article","created":{"date-parts":[[2024,6,12]],"date-time":"2024-06-12T14:02:16Z","timestamp":1718200936000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis"],"prefix":"10.1186","volume":"25","author":[{"given":"Hanna","family":"Jaula","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lauri","family":"Mattila","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisa","family":"Lappi-Blanco","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johanna","family":"Salonen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hannu","family":"V\u00e4h\u00e4nikkil\u00e4","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lauri","family":"Ahvenj\u00e4rvi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jukka S.","family":"Moilanen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Outi","family":"Kuismin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Terttu","family":"Harju","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riitta","family":"Kaarteenaho","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,6,12]]},"reference":[{"key":"2864_CR1","doi-asserted-by":"publisher","unstructured":"Zhang D, Newton CA. 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