{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T08:40:38Z","timestamp":1784882438384,"version":"3.55.0"},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,3,21]],"date-time":"2025-03-21T00:00:00Z","timestamp":1742515200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,3,21]],"date-time":"2025-03-21T00:00:00Z","timestamp":1742515200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"The SENSCIS and SENSCIS-ON trials were supported by Boehringer Ingelheim."}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:p>We used data from the SENSCIS and SENSCIS-ON trials to assess decline in forced vital capacity (FVC) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) who received long-term treatment with nintedanib and the effect of switching patients from placebo to nintedanib. In the SENSCIS trial, patients were randomised to receive nintedanib or placebo until the last patient reached week 52 but for \u2264\u2009100 weeks. In SENSCIS-ON, the extension to SENSCIS, all patients received open-label nintedanib. Per protocol, the off-treatment period between these trials was \u2264\u200912 weeks. We assessed the trajectory of FVC in patients who received nintedanib in SENSCIS and continued nintedanib in SENSCIS-ON (<jats:italic>n<\/jats:italic>\u2009=\u2009197) and in patients who received placebo in SENSCIS and initiated nintedanib in SENSCIS-ON (<jats:italic>n<\/jats:italic>\u2009=\u2009231). The last on-treatment measurement in SENSCIS and the baseline measurement of SENSCIS-ON were considered anchor measurements. In patients who received nintedanib in SENSCIS, the mean decline in FVC in the 52 weeks prior to the last on-treatment measurement in SENSCIS was \u2212\u200941.5 mL and the mean decline in FVC from baseline to week 52 of SENSCIS-ON was \u2212\u200958.3 mL. In patients who received placebo in SENSCIS, the mean decline in FVC in the 52 weeks prior to the last on-treatment measurement in SENSCIS was \u2212\u200996.8 mL and the mean decline in FVC from baseline to week 52 of SENSCIS-ON (when patients received nintedanib) was \u2212\u200942.8 mL. These findings illustrate the progressive nature of SSc-ILD and support the efficacy of nintedanib in slowing decline in lung function over the long term.<\/jats:p>","DOI":"10.1186\/s13075-025-03524-9","type":"journal-article","created":{"date-parts":[[2025,3,21]],"date-time":"2025-03-21T08:23:56Z","timestamp":1742545436000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Trajectories of forced vital capacity in patients with systemic sclerosis-associated interstitial lung disease"],"prefix":"10.1186","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0546-8310","authenticated-orcid":false,"given":"Oliver","family":"Distler","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madelon C","family":"Vonk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0506-9966","authenticated-orcid":false,"given":"Arata","family":"Azuma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5070-2535","authenticated-orcid":false,"given":"Maureen D.","family":"Mayes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh","family":"Khanna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristin B.","family":"Highland","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerrit","family":"Toenges","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarida","family":"Alves","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6149-0002","authenticated-orcid":false,"given":"Yannick","family":"Allanore","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,3,21]]},"reference":[{"key":"3524_CR1","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1164\/rccm.201903-0486OC","volume":"200","author":"AM Hoffmann-Vold","year":"2019","unstructured":"Hoffmann-Vold AM, Fretheim H, Halse AK, et al. Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med. 2019;200:1258\u201366.","journal-title":"Am J Respir Crit Care Med"},{"issue":"23","key":"3524_CR2","first-page":"05274","volume":"S0012\u20133692","author":"P Moinzadeh","year":"2023","unstructured":"Moinzadeh P, Bonella F, Oberste M, et al. Impact of systemic sclerosis-associated interstitial lung disease with and without pulmonary hypertension on survival: a large cohort study of the German network for systemic sclerosis. Chest. 2023;S0012\u20133692(23):05274\u20131.","journal-title":"Chest"},{"key":"3524_CR3","doi-asserted-by":"crossref","first-page":"E71","DOI":"10.1016\/S2665-9913(19)30144-4","volume":"2","author":"AM Hoffmann-Vold","year":"2020","unstructured":"Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheumatol. 2020;2:E71\u201383.","journal-title":"Lancet Rheumatol"},{"key":"3524_CR4","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1186\/s12931-022-02292-3","volume":"24","author":"FF Rahaghi","year":"2023","unstructured":"Rahaghi FF, Hsu VM, Kaner RJ, et al. Expert consensus on the management of systemic sclerosis-associated interstitial lung disease. Respir Res. 2023;24:6.","journal-title":"Respir Res"},{"key":"3524_CR5","doi-asserted-by":"crossref","first-page":"1670","DOI":"10.1002\/art.40130","volume":"69","author":"NS Goh","year":"2017","unstructured":"Goh NS, Hoyles RK, Denton CP, et al. Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol. 2017;69:1670\u20138.","journal-title":"Arthritis Rheumatol"},{"key":"3524_CR6","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1136\/annrheumdis-2018-213708","volume":"78","author":"ER Volkmann","year":"2019","unstructured":"Volkmann ER, Tashkin DP, Sim M, et al. Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts. Ann Rheum Dis. 2019;78:122\u201330.","journal-title":"Ann Rheum Dis"},{"key":"3524_CR7","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1186\/s13075-021-02710-9","volume":"24","author":"M Kreuter","year":"2022","unstructured":"Kreuter M, Del Galdo F, Miede C, et al. Impact of lung function decline on time to hospitalisation events in systemic sclerosis-associated interstitial lung disease (SSc-ILD): a joint model analysis. Arthritis Res Ther. 2022;24:19.","journal-title":"Arthritis Res Ther"},{"key":"3524_CR8","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1111\/resp.14579","volume":"28","author":"TM Maher","year":"2023","unstructured":"Maher TM, Stowasser S, Voss F, et al. Decline in forced vital capacity as a surrogate for mortality in patients with pulmonary fibrosis. Respirology. 2023;28:1147\u201353.","journal-title":"Respirology"},{"key":"3524_CR9","doi-asserted-by":"crossref","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","volume":"380","author":"O Distler","year":"2019","unstructured":"Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019;380:2518\u201328.","journal-title":"N Engl J Med"},{"key":"3524_CR10","doi-asserted-by":"crossref","first-page":"1722","DOI":"10.1136\/ard-2022-222564","volume":"81","author":"Y Allanore","year":"2022","unstructured":"Allanore Y, Vonk MC, Distler O, et al. Continued treatment with nintedanib in patients with systemic sclerosis-associated interstitial lung disease: data from SENSCIS-ON. Ann Rheum Dis. 2022;81:1722\u20139.","journal-title":"Ann Rheum Dis"},{"key":"3524_CR11","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1183\/09031936.05.00034805","volume":"26","author":"MR Miller","year":"2005","unstructured":"Miller MR, Hankinson J, Brusasco V, et al. Standardisation of spirometry. Eur Respir J. 2005;26:319\u201338.","journal-title":"Eur Respir J"},{"key":"3524_CR12","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1002\/art.41576","volume":"73","author":"TM Maher","year":"2021","unstructured":"Maher TM, Mayes MD, Kreuter M, et al. Effect of nintedanib on lung function in patients with systemic sclerosis-associated interstitial lung disease: further analyses of a randomized, double-blind, placebo-controlled trial. 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ACR Open Rheumatol. 2022;4:837\u201344.","journal-title":"ACR Open Rheumatol"},{"key":"3524_CR15","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1164\/rccm.202306-1113ST","volume":"209","author":"G Raghu","year":"2024","unstructured":"Raghu G, Montesi SB, Silver RM, et al. Treatment of systemic sclerosis-associated interstitial lung disease: evidence-based recommendations. An official American thoracic society clinical practice guideline. Am J Respir Crit Care Med. 2024;209:137\u201352.","journal-title":"Am J Respir Crit Care Med"},{"key":"3524_CR16","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1002\/acr.25347","volume":"76","author":"SR Johnson","year":"2024","unstructured":"Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American college of rheumatology (ACR)\/American college of chest physicians (CHEST) guideline for the screening and monitoring of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). 2024;76:1070\u201382.","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"3524_CR17","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1002\/acr.25348","volume":"76","author":"SR Johnson","year":"2024","unstructured":"Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American college of rheumatology (ACR)\/American college of chest physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). 2024;76:1051\u201369.","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"3524_CR18","doi-asserted-by":"crossref","first-page":"e18","DOI":"10.1164\/rccm.202202-0399ST","volume":"205","author":"G Raghu","year":"2022","unstructured":"Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS\/ERS\/JRS\/ALAT clinical practice guideline. 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Autoimmun Rev 2023;103463.","DOI":"10.1016\/j.autrev.2023.103463"}],"container-title":["Arthritis Research &amp; Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-025-03524-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13075-025-03524-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-025-03524-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,3,21]],"date-time":"2025-03-21T08:24:40Z","timestamp":1742545480000},"score":1,"resource":{"primary":{"URL":"https:\/\/arthritis-research.biomedcentral.com\/articles\/10.1186\/s13075-025-03524-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,21]]},"references-count":19,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["3524"],"URL":"https:\/\/doi.org\/10.1186\/s13075-025-03524-9","relation":{},"ISSN":["1478-6362"],"issn-type":[{"value":"1478-6362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,21]]},"assertion":[{"value":"7 October 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 March 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 March 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The study protocol was reviewed and approved by an Independent Ethics Committee and\/or Institutional Review Board at each trial site, as listed in the primary publications on the results of these trials [, ]. Written informed consent was obtained from all subjects before study entry.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"Oliver Distler has\/had consultancy relationship with and\/or has received research funding from and\/or has served as a speaker for the following companies in the area of potential treatments for SSc and its complications in the last three years: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Argenx, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Cantargia, Catalyze Capital, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck Sharp & Dohme, Miltenyi Biotec, Mitsubishi Tanabe, Nkarta, Novartis, Orion, Prometheus, Redx, Roivant, EMD Serono, Topadur, UCB; he has a patent issued \u201cmir-29 for the treatment of systemic sclerosis\u201d (US8247389, EP2331143) and is a co-founder of CITUS AG. Madelon C Vonk reports research support from Boehringer Ingelheim, Ferrer, Galapagos, Janssen; she acts as a speaker for Boehringer Ingelheim, Janssen, Merck Sharp & Dohme, Novartis and a board member for EUSTAR and Systemic Sclerosis ERN ReCONNET. Arata Azuma reports acting as a consultant for Boehringer Ingelheim, Kyorin Pharma, Taiho, Toray and acting as a speaker and receiving research support from Boehringer Ingelheim. Maureen D Mayes has received research support from Boehringer Ingelheim, Corbus, Eicos, Horizon Pharma, Mitsubishi Tanabe, Prometheus and royalties from the British Medical Journal, Oxford University Press and Springer International Publishing; she has acted as a speaker for Medtelligence and as an advisor or review panel member for Boehringer Ingelheim, EICOS, Mitsubishi Tanabe. Dinesh Khanna has acted as a consultant for AstraZeneca, Boehringer Ingelheim, Bristol Myers Squibb, CSL Behring, Genentech, Horizon Therapeutics, Janssen, Prometheus, UCB; as a speaker for Jannsen; and as a member of a data safety monitoring board for AbbVie; he has received research support from Bristol Myers Squibb, Horizon Therapeutics, Pfizer and royalties for the University of California Los Angeles Scleroderma Clinical Trials Consortium (SCTC) Gastrointestinal Tract instrument 2.0. Kristin B Highland has acted as a consultant and speaker for and received research support from Boehringer Ingelheim and acted as an advisor or review panel member for the Scleroderma Foundation. Gerrit Toenges and Margarida Alves are employees of Boehringer Ingelheim. Yannick Allanore has acted as an advisor or review panel member for AstraZeneca, Boehringer Ingelheim, Chemomab, Curzion, Medsenic, Menarini, Prometheus, Sanofi; as a consultant for Boehringer Ingelheim and Sanofi; and as a speaker for AbbVie, Boehringer Ingelheim, Janssen.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"63"}}