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Randomized Controlled Trial
. 2025 Mar 21;27(1):63.
doi: 10.1186/s13075-025-03524-9.

Trajectories of forced vital capacity in patients with systemic sclerosis-associated interstitial lung disease

Oliver Distler  1 , Madelon C Vonk  2 , Arata Azuma  3   4 , Maureen D Mayes  5 , Dinesh Khanna  6 , Kristin B Highland  7 , Gerrit Toenges  8 , Margarida Alves  9 , Yannick Allanore  10
Affiliations
Randomized Controlled Trial

Trajectories of forced vital capacity in patients with systemic sclerosis-associated interstitial lung disease

Oliver Distler et al. Arthritis Res Ther. .

Abstract

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 ≤ 100 weeks. In SENSCIS-ON, the extension to SENSCIS, all patients received open-label nintedanib. Per protocol, the off-treatment period between these trials was ≤ 12 weeks. We assessed the trajectory of FVC in patients who received nintedanib in SENSCIS and continued nintedanib in SENSCIS-ON (n = 197) and in patients who received placebo in SENSCIS and initiated nintedanib in SENSCIS-ON (n = 231). 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 - 41.5 mL and the mean decline in FVC from baseline to week 52 of SENSCIS-ON was - 58.3 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 - 96.8 mL and the mean decline in FVC from baseline to week 52 of SENSCIS-ON (when patients received nintedanib) was - 42.8 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.

Keywords: Disease progression; Pulmonary fibrosis; Pulmonary function tests; Systemic scleroderma.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: 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 [9, 10]. Written informed consent was obtained from all subjects before study entry. Consent for publication: Not applicable. Competing interests: 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 “mir-29 for the treatment of systemic sclerosis” (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.

Figures

Fig. 1
Fig. 1
Measuring the trajectories of FVC in SENSCIS and SENSCIS-ON
Fig. 2
Fig. 2
Trajectories of FVC in SENSCIS and SENSCIS-ON in (a) patients who received nintedanib in SENSCIS and (b) patients who received placebo in SENSCIS
See this image and copyright information in PMC

References

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