{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T20:28:13Z","timestamp":1790368093676,"version":"4.1.0"},"reference-count":31,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,3,23]],"date-time":"2025-03-23T00:00:00Z","timestamp":1742688000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/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":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background and Objective<\/jats:title><jats:p>Real\u2010world data on lung function course of patients with progressive pulmonary fibrosis (PPF) treated with anti\u2010fibrotic medication are limited. We evaluated forced vital capacity (FVC) decline in patients with PPF and idiopathic pulmonary fibrosis (IPF) who started anti\u2010fibrotic treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This was a nationwide multi\u2010centre registry study in 16 hospitals throughout the Netherlands. Patients treated with anti\u2010fibrotic medication, with at least two in\u2010hospital pulmonary function tests before and after the initiation of anti\u2010fibrotic treatment, were included. Linear mixed\u2010effects modelling was used to analyse lung function trajectories 1 year before and after the start of anti\u2010fibrotic treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Data from 538 patients (<jats:italic>n\u2009=<\/jats:italic>\u2009142 with PPF, <jats:italic>n\u2009=<\/jats:italic>\u2009396 with IPF) were analysed. In PPF, the mean annualised FVC decline was 412\u2009mL (95% confidence interval [CI]: 308\u2013517\u2009mL) before the initiation of anti\u2010fibrotic treatment, and 18\u2009mL (95% CI: 9\u2013124\u2009mL) in the first year after. The corresponding declines for IPF were 158\u2009mL (95% CI: 78\u2013239\u2009mL) and 38\u2009mL (95% CI: 24\u2013101\u2009mL). In both groups, treatment significantly slowed down FVC decline, although the change was larger in the PPF group (<jats:italic>p<\/jats:italic>\u2009=\u20090.0006). In the first year after treatment initiation, 23.9% of patients with PPF and 28.0% with IPF had disease progression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The FVC decline significantly slowed after the initiation of treatment for both IPF and PPF. Nevertheless, a significant proportion of patients exhibited disease progression, despite the start of anti\u2010fibrotic treatment. Early identification of these patients is crucial for treatment adaptations and inclusion in clinical trials.<\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.70030","type":"journal-article","created":{"date-parts":[[2025,3,24]],"date-time":"2025-03-24T00:30:01Z","timestamp":1742776201000},"page":"417-423","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Lung Function Course of Patients With Pulmonary Fibrosis After Initiation of Anti\u2010Fibrotic Treatment: Real\u2010World Data From the Dutch National Registry"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5541-5092","authenticated-orcid":false,"given":"Mark G. J. P.","family":"Platenburg","sequence":"first","affiliation":[{"name":"ILD Centre of Excellence, Member of European Reference Network\u2010Lung St Antonius Hospital  Nieuwegein the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-7499-5926","authenticated-orcid":false,"given":"Gizal","family":"Nakshbandi","sequence":"additional","affiliation":[{"name":"Centre of Excellence for ILD and Sarcoidosis, Department of Respiratory Medicine Erasmus Medical Centre  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catharina C.","family":"Moor","sequence":"additional","affiliation":[{"name":"Centre of Excellence for ILD and Sarcoidosis, Department of Respiratory Medicine Erasmus Medical Centre  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aernoud A.","family":"van Batenburg","sequence":"additional","affiliation":[{"name":"ILD Centre of Excellence, Member of European Reference Network\u2010Lung St Antonius Hospital  Nieuwegein the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R\u00e9my L. M.","family":"Mostard","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Zuyderland Medical Centre  Heerlen the Netherlands"},{"name":"Department of Respiratory Medicine Maastricht University Medical Centre (MUMC+)  Maastricht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mareye","family":"Voortman","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Division of Heart &amp; Lungs University Medical Centre Utrecht  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda A.A.","family":"Moonen","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine Rijnstate Hospital  Arnhem the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolle","family":"Hekelaar","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine Medisch Spectrum Twente  Enschede the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria J.","family":"Overbeek","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine Haaglanden Medical Centre  The Hague the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"A.H.A. Bogaarts","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Diseases VieCuri Medical Centre  Venlo the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henk","family":"Kramer","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine Martini Hospital  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emiel. R.","family":"Marges","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Leiden University Medical Centre  Leiden the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bart B.","family":"Boerrigter","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine, Centre of Excellence for Interstitial Lung Diseases and Sarcoidosis Amsterdam University Medical Centre, VUMC  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Bresser","sequence":"additional","affiliation":[{"name":"ILD Centre of Excellence, Department of Respiratory Medicine OLVG  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eveline L.","family":"Schakenraad","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine Catharina Hospital  Eindhoven the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"van der Maten","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine Medical Centre Leeuwarden  Leeuwarden the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niels C.A.","family":"van der Sloot","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Medicine Jeroen Bosch Hospital  Hertogenbosch the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Walen","sequence":"additional","affiliation":[{"name":"Department of Pulmonology Isala  Zwolle the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro Miranda","family":"Afonso","sequence":"additional","affiliation":[{"name":"Department of Biostatistics Erasmus Medical Centre  Rotterdam the Netherlands"},{"name":"Department of Epidemiology Erasmus Medical Centre  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4527-6962","authenticated-orcid":false,"given":"Marlies S.","family":"Wijsenbeek","sequence":"additional","affiliation":[{"name":"Centre of Excellence for ILD and Sarcoidosis, Department of Respiratory Medicine Erasmus Medical Centre  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan C.","family":"Grutters","sequence":"additional","affiliation":[{"name":"ILD Centre of Excellence, Member of European Reference Network\u2010Lung St Antonius Hospital  Nieuwegein the Netherlands"},{"name":"Department of Pulmonology, Division of Heart &amp; Lungs University Medical Centre Utrecht  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,3,23]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00957-2022"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1705751"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202202-0399ST"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(22)00260-0"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(20)30554-3"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908681"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1903076"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(19)30341-8"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(16)30152-7"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2016.10.029"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(22)00359-9"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00423-2022"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-023-06689-3"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp\u20102020\u2010000782"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm9113763"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-019-1182-z"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-019-1043-9"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-023-45027-0"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12890\u2010023\u201002336\u20104"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keae110"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.04503-2020"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201711-2174OC"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.3390\/jpm13091307"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(18)30339-4"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1402584"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1159\/000363064"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.04587-2020"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp\u20102022\u2010001580"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp-2021-001026"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(19)30255-3"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.04587-2020"}],"container-title":["Respirology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/resp.70030","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,27]],"date-time":"2025-06-27T06:54:32Z","timestamp":1751007272000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/resp.70030"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,23]]},"references-count":31,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,5]]}},"alternative-id":["10.1111\/resp.70030"],"URL":"https:\/\/doi.org\/10.1111\/resp.70030","archive":["Portico"],"relation":{},"ISSN":["1323-7799","1440-1843"],"issn-type":[{"value":"1323-7799","type":"print"},{"value":"1440-1843","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,23]]},"assertion":[{"value":"2024-07-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-02-26","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-03-23","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}