{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T10:52:15Z","timestamp":1781866335176,"version":"3.54.5"},"reference-count":30,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T00:00:00Z","timestamp":1781740800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background and Objectives: Nintedanib is indicated for progressive pulmonary fibrosis (PPF) based on clinical trial results. The primary objectives of this study were to evaluate the effectiveness of nintedanib on forced vital capacity (FVC) and diffusing lung capacity for CO (DLCO) after one year of treatment, and to compare the annual rate of decline (\u201cslope\u201d) for FVC, DLCO and 6 minute walking distance (6MWD) with the year prior to treatment. The secondary objectives were antifibrotic safety, tolerability, adverse events, immunosuppressant use, dyspnoea and survival. Materials and Methods: This study was a single-centre, retrospective, observational cohort study that included consecutive patients with PPF treated with nintedanib. Results: Fifty-five patients with non-IPF fibrotic ILD initiated nintedanib due to fibrosis progression. Most patients (63.4%) stabilised\/improved FVC after 1 year of treatment, and 82.5% stabilised\/improved DLCO. The \u201cslope\u201d of FVC and DLCO was reduced after 1 year of treatment compared to the year before initiation, although the difference was not statistically significant: FVC slope was +0.61% in the year after initiation vs. \u22122.3% in the year prior (mean change: 2.94%, 95%CI [\u22124.74, 10.62]); DLCO slope was \u22123.8% after treatment vs. \u22127% before initiation (mean change: 3.24%, 95%CI [\u22127.43, 13.92]). Dyspnoea improved in 23.2% of patients. A reduction in immunosuppressant use was observed after nintedanib initiation. Forty-one patients (74.5%) experienced at least one side effect: diarrhoea (60%), hepatotoxicity (23.6%), or asthenia (12.7%). Fifteen patients required permanent or temporary treatment discontinuation. Conclusions: In our real-world PPF cohort, most patients showed FVC and\/or DLCO stabilisation or improvement after one year of nintedanib treatment. A non-significant reduction in the rate of FVC decline after one year of treatment was also observed, as was a reduction in symptom severity in our real-life PPF cohort.<\/jats:p>","DOI":"10.3390\/medicina62061187","type":"journal-article","created":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T09:18:43Z","timestamp":1781860723000},"page":"1187","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Real-World Experience with Nintedanib in a Progressive Pulmonary Fibrosis Cohort"],"prefix":"10.3390","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0940-3215","authenticated-orcid":false,"given":"Vanesa","family":"Vicens-Zygmunt","sequence":"first","affiliation":[{"name":"Interstitial Lung Disease (ILD) Unit, Pulmonology Department, University Hospital of Bellvitge, Institute of Research of Bellvitge (DIBELL), University of Barcelona (UB), Biomedical Research Network Center Respiratory Diseases (CIBERES), 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4027-8907","authenticated-orcid":false,"given":"Jaume","family":"Bordas-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Interstitial Lung Disease (ILD) Unit, Pulmonology Department, University Hospital of Bellvitge, Institute of Research of Bellvitge (DIBELL), University of Barcelona (UB), Biomedical Research Network Center Respiratory Diseases (CIBERES), 08907 Barcelona, Spain"},{"name":"Interstitial Lung Disease (ILD) Unit, Pulmonology Department, Hospital General de Granollers, 08402 Granollers, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-0271-1767","authenticated-orcid":false,"given":"Miriam","family":"Mu\u00f1oz-Bola\u00f1o","sequence":"additional","affiliation":[{"name":"Pharmacy Department, University Hospital of Bellvitge, 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o","family":"Carmezim","sequence":"additional","affiliation":[{"name":"Biostatistics Unit (UBiDi), Bellvitge Biomedical Research Institute (IDIBELL), 08840 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0467-4024","authenticated-orcid":false,"given":"Ana Bel\u00e9n","family":"Llanos-Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Interstitial Lung Disease Unit, Pulmonology Department, University Complex Hospital of Canarias, 38320 Santa Cruz de Tenerife, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8736-0671","authenticated-orcid":false,"given":"Adri\u00e0","family":"Domingo-Carnice","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Department, University Hospital of Bellvitge, 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dolores","family":"Rodr\u00edguez-Cumplido","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Department, University Hospital of Bellvitge, 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2010-7679","authenticated-orcid":false,"given":"Guadalupe","family":"Bermudo-Peloche","sequence":"additional","affiliation":[{"name":"Interstitial Lung Disease (ILD) Unit, Pulmonology Department, University Hospital of Bellvitge, Institute of Research of Bellvitge (DIBELL), University of Barcelona (UB), Biomedical Research Network Center Respiratory Diseases (CIBERES), 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristian","family":"Teb\u00e9-Cordom\u00ed","sequence":"additional","affiliation":[{"name":"Biostatistics Unit (UBiDi), Bellvitge Biomedical Research Institute (IDIBELL), 08840 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Pe\u00f1afiel","sequence":"additional","affiliation":[{"name":"Biostatistics Unit (UBiDi), Bellvitge Biomedical Research Institute (IDIBELL), 08840 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roser","family":"Llop-Rius","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Department, University Hospital of Bellvitge, 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Molina-Molina","sequence":"additional","affiliation":[{"name":"Interstitial Lung Disease (ILD) Unit, Pulmonology Department, University Hospital of Bellvitge, Institute of Research of Bellvitge (DIBELL), University of Barcelona (UB), Biomedical Research Network Center Respiratory Diseases (CIBERES), 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","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. 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