Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone
Background There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed wit...
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| creator | Májek, Ondrej Gregor, Jakub Mogulkoc, Nesrin Lewandowska, Katarzyna Sterclová, Martina Müller, Veronika Hájková, Marta Kramer, Mordechai R Tekavec-Trkanjec, Jasna Jovanovic, Dragana Studnicka, Michael Stoeva, Natalia Kirchgässler, Klaus-Uwe Littnerová, Simona Dusek, Ladislav Vasáková, Martina Koziar |
| description | Background There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. Methods and findings We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. Results A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. Conclusions This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF. |
| doi_str_mv | 10.1371/journal.pone.0273854 |
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We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. Methods and findings We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. Results A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. Conclusions This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0273854</identifier><identifier>PMID: 36048805</identifier><language>eng</language><publisher>San Francisco: Public Library of Science</publisher><subject>Biology and Life Sciences ; Biopsy ; Care and treatment ; Diagnosis ; Dyspnea ; Fibrosis ; Histopathology ; Lung diseases ; Lung transplants ; Lungs ; Medical diagnosis ; Medical prognosis ; Medicine and Health Sciences ; Patients ; Pulmonary fibrosis ; Research and Analysis Methods ; Respiratory function ; Statistical analysis ; Survival</subject><ispartof>PloS one, 2022-09, Vol.17 (9), p.e0273854-e0273854</ispartof><rights>COPYRIGHT 2022 Public Library of Science</rights><rights>2022 Májek et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. 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We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. Methods and findings We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. Results A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. Conclusions This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.</description><subject>Biology and Life Sciences</subject><subject>Biopsy</subject><subject>Care and treatment</subject><subject>Diagnosis</subject><subject>Dyspnea</subject><subject>Fibrosis</subject><subject>Histopathology</subject><subject>Lung diseases</subject><subject>Lung transplants</subject><subject>Lungs</subject><subject>Medical diagnosis</subject><subject>Medical prognosis</subject><subject>Medicine and Health Sciences</subject><subject>Patients</subject><subject>Pulmonary fibrosis</subject><subject>Research and Analysis Methods</subject><subject>Respiratory function</subject><subject>Statistical 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and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone</title><author>Májek, Ondrej ; Gregor, Jakub ; Mogulkoc, Nesrin ; Lewandowska, Katarzyna ; Sterclová, Martina ; Müller, Veronika ; Hájková, Marta ; Kramer, Mordechai R ; Tekavec-Trkanjec, Jasna ; Jovanovic, Dragana ; Studnicka, Michael ; Stoeva, Natalia ; Kirchgässler, Klaus-Uwe ; Littnerová, Simona ; Dusek, Ladislav ; Vasáková, Martina Koziar</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c669t-57a2ae592ae011da6987df532b6ebdd55977432397303e716d919d0afb4c4d9c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Biology and Life Sciences</topic><topic>Biopsy</topic><topic>Care and treatment</topic><topic>Diagnosis</topic><topic>Dyspnea</topic><topic>Fibrosis</topic><topic>Histopathology</topic><topic>Lung diseases</topic><topic>Lung transplants</topic><topic>Lungs</topic><topic>Medical diagnosis</topic><topic>Medical prognosis</topic><topic>Medicine and Health Sciences</topic><topic>Patients</topic><topic>Pulmonary fibrosis</topic><topic>Research and Analysis Methods</topic><topic>Respiratory function</topic><topic>Statistical analysis</topic><topic>Survival</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Májek, Ondrej</creatorcontrib><creatorcontrib>Gregor, Jakub</creatorcontrib><creatorcontrib>Mogulkoc, Nesrin</creatorcontrib><creatorcontrib>Lewandowska, Katarzyna</creatorcontrib><creatorcontrib>Sterclová, Martina</creatorcontrib><creatorcontrib>Müller, Veronika</creatorcontrib><creatorcontrib>Hájková, Marta</creatorcontrib><creatorcontrib>Kramer, Mordechai R</creatorcontrib><creatorcontrib>Tekavec-Trkanjec, Jasna</creatorcontrib><creatorcontrib>Jovanovic, Dragana</creatorcontrib><creatorcontrib>Studnicka, Michael</creatorcontrib><creatorcontrib>Stoeva, 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Marta</au><au>Kramer, Mordechai R</au><au>Tekavec-Trkanjec, Jasna</au><au>Jovanovic, Dragana</au><au>Studnicka, Michael</au><au>Stoeva, Natalia</au><au>Kirchgässler, Klaus-Uwe</au><au>Littnerová, Simona</au><au>Dusek, Ladislav</au><au>Vasáková, Martina Koziar</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone</atitle><jtitle>PloS one</jtitle><date>2022-09-01</date><risdate>2022</risdate><volume>17</volume><issue>9</issue><spage>e0273854</spage><epage>e0273854</epage><pages>e0273854-e0273854</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>Background There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. Methods and findings We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. Results A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. Conclusions This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.</abstract><cop>San Francisco</cop><pub>Public Library of Science</pub><pmid>36048805</pmid><doi>10.1371/journal.pone.0273854</doi><tpages>e0273854</tpages><orcidid>https://orcid.org/0000-0002-0034-3084</orcidid><orcidid>https://orcid.org/0000-0003-2431-7873</orcidid><orcidid>https://orcid.org/0000-0002-0424-9941</orcidid><oa>free_for_read</oa></addata></record> |
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| recordid | cdi_plos_journals_2708995765 |
| source | DOAJ Directory of Open Access Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central; Free Full-Text Journals in Chemistry; Public Library of Science (PLoS) |
| subjects | Biology and Life Sciences Biopsy Care and treatment Diagnosis Dyspnea Fibrosis Histopathology Lung diseases Lung transplants Lungs Medical diagnosis Medical prognosis Medicine and Health Sciences Patients Pulmonary fibrosis Research and Analysis Methods Respiratory function Statistical analysis Survival |
| title | Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-03T21%3A07%3A35IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Survival%20and%20lung%20function%20decline%20in%20patients%20with%20definite,%20probable%20and%20possible%20idiopathic%20pulmonary%20fibrosis%20treated%20with%20pirfenidone&rft.jtitle=PloS%20one&rft.au=M%C3%A1jek,%20Ondrej&rft.date=2022-09-01&rft.volume=17&rft.issue=9&rft.spage=e0273854&rft.epage=e0273854&rft.pages=e0273854-e0273854&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0273854&rft_dat=%3Cgale_plos_%3EA715828210%3C/gale_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2708995765&rft_id=info:pmid/36048805&rft_galeid=A715828210&rft_doaj_id=oai_doaj_org_article_62d03fd7fced4bb68c72565e31432e8a&rfr_iscdi=true |