{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T18:42:20Z","timestamp":1785868940303,"version":"3.56.0"},"reference-count":33,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T00:00:00Z","timestamp":1677456000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Connective tissue diseases-associated interstitial lung disease (CTD-ILD) is a heterogeneous condition that impairs quality of life and is associated with premature death. Progressive pulmonary fibrosis (PPF) has been identified as an important risk factor for poor prognosis. However, different criteria for PPF are used in clinical studies, which may complicate comparison between trials and translation of study findings into clinical practice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a retrospective single center study in patients with CTD-ILD. The prognostic relevance of PPF definitions, including INBUILD, ATS\/ERS\/JRS\/ALAT 2022, and simplified progressive fibrosing (simplified PF) criteria, were examined in this cohort and validated in the other reported Dutch CTD-ILD cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 230 patients with CTD-ILD were included and the median follow-up period was six (3\u20149) years. Mortality risk was independently associated with age (adjusted HR 1.07, <jats:italic>p<\/jats:italic>\u2009&amp;lt;\u20090.001), smoking history (adjusted HR 1.90, <jats:italic>p<\/jats:italic>\u2009=\u20090.045), extent of fibrosis on high-resolution computed tomography (HRCT) at baseline (adjusted HR 1.05, <jats:italic>p<\/jats:italic>\u2009=\u20090.018) and baseline DLCO (adjusted HR 0.97, <jats:italic>p<\/jats:italic>\u2009=\u20090.013). Patients with regular pulmonary function tests in the first 2 years (adjusted HR 0.42, <jats:italic>p<\/jats:italic>\u2009=\u20090.002) had a better survival. The prognostic relevance for survival was similar between the three PPF criteria in the two cohorts.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Higher age, smoking, increased extent of fibrosis and low baseline DLCO were associated with poor prognosis, while regular pulmonary function evaluation was associated with better survival. The INBUILD, ATS\/ERS\/JRS\/ALAT 2022, and simplified PF criteria revealed similar prognostication.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fmed.2023.1106560","type":"journal-article","created":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T04:44:03Z","timestamp":1677473043000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":16,"title":["Prognostication of progressive pulmonary fibrosis in connective tissue disease-associated interstitial lung diseases: A cohort study"],"prefix":"10.3389","volume":"10","author":[{"given":"Yu-Hsiang","family":"Chiu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maaike F. M.","family":"Koops","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mareye","family":"Voortman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. Wouter","family":"van Es","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucianne C. M.","family":"Langezaal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paco M. J.","family":"Welsing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Jamnitski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne E.","family":"Wind","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacob M.","family":"van Laar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan C.","family":"Grutters","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Spierings","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,2,27]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"180076","DOI":"10.1183\/16000617.0076-2018","article-title":"Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases","volume":"27","author":"Cottin","year":"2018","journal-title":"Eur Respir Rev"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"480","DOI":"10.1016\/j.semarthrit.2020.01.006","article-title":"Circulating lung biomarkers in idiopathic lung fibrosis and interstitial lung diseases associated with connective tissue diseases: where do we stand?","volume":"50","author":"Elhai","year":"2020","journal-title":"Semin Arthritis Rheum"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"106579","DOI":"10.1016\/j.rmed.2021.106579","article-title":"Predictors for progressive fibrosis in patients with connective tissue disease associated interstitial lung diseases","volume":"187","author":"Chiu","year":"2021","journal-title":"Respir Med"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1136\/annrheumdis-2020-217230","article-title":"Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs)","volume":"80","author":"Spagnolo","year":"2021","journal-title":"Ann Rheum Dis"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"2168","DOI":"10.3899\/jrheum.141182","article-title":"Connective tissue disease-associated interstitial lung diseases (CTD-ILD) \u2013 report from OMERACT CTD-ILD working group","volume":"42","author":"Khanna","year":"2015","journal-title":"J Rheumatol"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"1670","DOI":"10.1002\/art.40130","article-title":"Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis","volume":"69","author":"Goh","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"e18","DOI":"10.1164\/rccm.202202-0399ST","article-title":"Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS\/ERS\/JRS\/ALAT clinical practice guideline","volume":"205","author":"Raghu","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"1718","DOI":"10.1056\/NEJMoa1908681","article-title":"Nintedanib in progressive fibrosing interstitial lung diseases","volume":"381","author":"Flaherty","year":"2019","journal-title":"N Engl J Med"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1016\/S2213-2600(20)30554-3","article-title":"Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial","volume":"9","author":"Behr","year":"2021","journal-title":"Lancet Respir Med"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1016\/S2213-2600(17)30433-2","article-title":"Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner society white paper","volume":"6","author":"Lynch","year":"2018","journal-title":"Lancet Respir Med"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"209","DOI":"10.3389\/fmed.2019.00209","article-title":"Interstitial pneumonia with autoimmune features (IPAF)","volume":"6","author":"Fernandes","year":"2019","journal-title":"Front Med (Lausanne)"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1016\/S0140-6736(12)61079-4","article-title":"Interstitial lung disease in connective tissue disorders","volume":"380","author":"Fischer","year":"2012","journal-title":"Lancet"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"788","DOI":"10.1164\/rccm.2009-040GL","article-title":"An official ATS\/ERS\/JRS\/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management","volume":"183","author":"Raghu","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"e44","DOI":"10.1164\/rccm.201807-1255ST","article-title":"Diagnosis of idiopathic pulmonary fibrosis. An official ATS\/ERS\/JRS\/ALAT clinical practice guideline","volume":"198","author":"Raghu","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"1338","DOI":"10.1164\/rccm.200611-1685OC","article-title":"Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project","volume":"177","author":"Travis","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1148\/radiol.2322031223","article-title":"CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia","volume":"232","author":"Desai","year":"2004","journal-title":"Radiology"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"224","DOI":"10.1007\/s13665-012-0028-7","article-title":"Connective tissue disease-associated interstitial lung disease: a review","volume":"1","author":"Gutsche","year":"2012","journal-title":"Curr Respir Care Rep"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1186\/s12890-022-02105-9","article-title":"Reticulation pattern without honeycombing on high-resolution CT is associated with the risk of disease progression in interstitial lung diseases","volume":"22","author":"Mononen","year":"2022","journal-title":"BMC Pulm Med"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"1248","DOI":"10.1164\/rccm.200706-877OC","article-title":"Interstitial lung disease in systemic sclerosis: a simple staging system","volume":"177","author":"Goh","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"2110","DOI":"10.21037\/apm-21-3974","article-title":"Patterns of lung fibrosis in patients with interstitial pneumonia with autoimmune features and connective tissue diseases-associated interstitial lung disease-a narrative review","volume":"11","author":"Rzepka-Wrona","year":"2022","journal-title":"Ann Palliat Med"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1186\/s12890-019-0943-2","article-title":"Demographic and clinical predictors of progression and mortality in connective tissue disease-associated interstitial lung disease: a retrospective cohort study","volume":"19","author":"Chan","year":"2019","journal-title":"BMC Pulm Med"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1183\/13993003.00357-2015","article-title":"Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease","volume":"47","author":"Solomon","year":"2016","journal-title":"Eur Respir J"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"996","DOI":"10.1016\/j.semarthrit.2021.07.002","article-title":"MUC5B promoter variant rs35705950 and rheumatoid arthritis associated interstitial lung disease survival and progression","volume":"51","author":"Juge","year":"2021","journal-title":"Semin Arthritis Rheum"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1136\/annrheumdis-2020-217455","article-title":"Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database","volume":"80","author":"Hoffmann-Vold","year":"2021","journal-title":"Ann Rheum Dis"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"542","DOI":"10.1002\/art.39971","article-title":"Progressive decline of lung function in rheumatoid arthritis-associated interstitial lung disease","volume":"69","author":"Zamora-Legoff","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"ref26","doi-asserted-by":"publisher","first-page":"347","DOI":"10.3390\/medicina57040347","article-title":"Management of myositis-associated interstitial lung disease","volume":"57","author":"Fujisawa","year":"2021","journal-title":"Medicina (Kaunas)"},{"key":"ref27","doi-asserted-by":"publisher","first-page":"e70","DOI":"10.1212\/WNL.0000000000009727","article-title":"Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: study of 121 cases","volume":"95","author":"Allenbach","year":"2020","journal-title":"Neurology"},{"key":"ref28","doi-asserted-by":"publisher","first-page":"2000085","DOI":"10.1183\/13993003.00085-2020","article-title":"The natural history of progressive fibrosing interstitial lung diseases","volume":"55","author":"Brown","year":"2020","journal-title":"Eur Respir J"},{"key":"ref29","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1136\/thoraxjnl-2015-207252","article-title":"Interobserver agreement for the ATS\/ERS\/JRS\/ALAT criteria for a UIP pattern on CT","volume":"71","author":"Walsh","year":"2016","journal-title":"Thorax"},{"key":"ref30","doi-asserted-by":"publisher","first-page":"100228","DOI":"10.1016\/j.ejro.2020.100228","article-title":"Interobserver variability in high-resolution CT of the lungs","volume":"7","author":"Widell","year":"2020","journal-title":"Eur J Radiol Open"},{"key":"ref31","first-page":"S26","article-title":"A computer-aided diagnosis system for quantitative scoring of extent of lung fibrosis in scleroderma patients","volume":"28","author":"Kim","year":"2010","journal-title":"Clin Exp Rheumatol"},{"key":"ref32","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1007\/s13665-022-00290-w","article-title":"Management of connective tissue disease-related interstitial lung disease","volume":"11","author":"Ahmed","year":"2022","journal-title":"Curr Pulmonol Rep"},{"key":"ref33","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1055\/s-0039-1684020","article-title":"Multidisciplinary evaluation in patients with lung disease associated with connective tissue disease","volume":"40","author":"Wells","year":"2019","journal-title":"Semin Respir Crit Care Med"}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2023.1106560\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T04:44:08Z","timestamp":1677473048000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2023.1106560\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,27]]},"references-count":33,"alternative-id":["10.3389\/fmed.2023.1106560"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2023.1106560","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,27]]},"article-number":"1106560"}}