{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T01:28:45Z","timestamp":1792114125796,"version":"4.4.0"},"reference-count":36,"publisher":"European Respiratory Society (ERS)","issue":"6","license":[{"start":{"date-parts":[[2020,3,26]],"date-time":"2020-03-26T00:00:00Z","timestamp":1585180800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001003","name":"Boehringer Ingelheim","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001003","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2020,2,20]]},"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:p>We used data from the INBUILD and INPULSIS trials to investigate the natural history of progressive fibrosing interstitial lung diseases (ILDs).<\/jats:p>\n                  <jats:p>\n                    Subjects in the two INPULSIS trials had a clinical diagnosis of idiopathic pulmonary fibrosis (IPF) while subjects in the INBUILD trial had a progressive fibrosing ILD other than IPF and met protocol-defined criteria for ILD progression despite management. Using data from the placebo groups, we compared the rate of decline in forced vital capacity (FVC) (mL\u00b7year\n                    <jats:sup>\u22121<\/jats:sup>\n                    ) and mortality over 52\u2005weeks in the INBUILD trial with pooled data from the INPULSIS trials.\n                  <\/jats:p>\n                  <jats:p>\n                    The adjusted mean annual rate of decline in FVC in the INBUILD trial (n=331) was similar to that observed in the INPULSIS trials (n=423) (\u2212192.9\u2005mL\u00b7year\n                    <jats:sup>\u22121<\/jats:sup>\n                    and \u2212221.0\u2005mL\u00b7year\n                    <jats:sup>\u22121<\/jats:sup>\n                    , respectively; nominal p-value=0.19). The proportion of subjects who had a relative decline in FVC &gt;10% predicted at Week 52 was 48.9% in the INBUILD trial and 48.7% in the INPULSIS trials, and the proportion who died over 52\u2005weeks was 5.1% in the INBUILD trial and 7.8% in the INPULSIS trials. A relative decline in FVC &gt;10% predicted was associated with an increased risk of death in the INBUILD trial (hazard ratio 3.64) and the INPULSIS trials (hazard ratio 3.95).\n                  <\/jats:p>\n                  <jats:p>These findings indicate that patients with fibrosing ILDs other than IPF, who are progressing despite management, have a subsequent clinical course similar to patients with untreated IPF, with a high risk of further ILD progression and early mortality.<\/jats:p>","DOI":"10.1183\/13993003.00085-2020","type":"journal-article","created":{"date-parts":[[2020,3,26]],"date-time":"2020-03-26T16:25:12Z","timestamp":1585239912000},"page":"2000085","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":243,"title":["The natural history of progressive fibrosing interstitial lung diseases"],"prefix":"10.1183","volume":"55","author":[{"given":"Kevin K.","family":"Brown","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernando J.","family":"Martinez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon L.F.","family":"Walsh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor J.","family":"Thannickal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7336-7458","authenticated-orcid":false,"given":"Antje","family":"Prasse","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rozsa","family":"Schlenker-Herceg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rainer-Georg","family":"Goeldner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Clerisme-Beaty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay","family":"Tetzlaff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-0955","authenticated-orcid":false,"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Athol U.","family":"Wells","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2020,3,26]]},"reference":[{"key":"2024102101255976000_55.6.2000085.1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201807-1255ST"},{"key":"2024102101255976000_55.6.2000085.2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201308-1483ST"},{"key":"2024102101255976000_55.6.2000085.3","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00692-2018"},{"key":"2024102101255976000_55.6.2000085.4","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0100-2018"},{"key":"2024102101255976000_55.6.2000085.5","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1186\/s12931-019-1022-1","article-title":"The natural history of progressive fibrosing interstitial lung diseases","volume":"20","author":"Kolb","year":"2019","journal-title":"Respir Res"},{"key":"2024102101255976000_55.6.2000085.6","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.med.55.091902.103810"},{"key":"2024102101255976000_55.6.2000085.7","doi-asserted-by":"publisher","DOI":"10.1002\/art.22559"},{"key":"2024102101255976000_55.6.2000085.8","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00003214"},{"key":"2024102101255976000_55.6.2000085.9","doi-asserted-by":"publisher","DOI":"10.1097\/BOR.0000000000000219"},{"key":"2024102101255976000_55.6.2000085.10","doi-asserted-by":"crossref","first-page":"1900161","DOI":"10.1183\/13993003.00161-2019","article-title":"Potential of nintedanib in treatment of progressive fibrosing interstitial lung diseases","volume":"54","author":"Wollin","year":"2019","journal-title":"Eur Respir J"},{"key":"2024102101255976000_55.6.2000085.11","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1097\/RCT.0b013e31823ed437","article-title":"Idiopathic nonspecific interstitial pneumonia: changes in high-resolution computed tomography on long-term follow-up","volume":"36","author":"Kim","year":"2012","journal-title":"J Comput Assist Tomogr"},{"key":"2024102101255976000_55.6.2000085.12","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1513\/AnnalsATS.201801-067OC","article-title":"Heterogeneity in unclassifiable interstitial lung disease. 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