Effects of nintedanib in patients with limited cutaneous systemic sclerosis and interstitial lung disease
Yannick Allanore, Dinesh Khanna, Vanessa Smith, Martin Aringer, Anna‐Maria Hoffmann‐Vold, Masataka Kuwana, Peter A. Merkel, Christian Stock, Steven Sambevski, Christopher P. Denton, SENSCIS Trial Investigators, Miguel Bergna, Gema Casado, P Mannucci Walter, Susanna Proudman, Wendy Stevens, Vivek Thakkar, Lauren Troy, J Loeffler-Ragg, Horst Olschewski, Béatrice André, Benjamin Bondue, Frédéric Houssiau, V. Smith, Wim Wuyts, Valderílio Feijó Azevedo, Sindhu R. Johnson, EC Keystone, Nader Khalidi, Marc C. Levesque, R. Maturana Rozas, A. Silva Orellana, Chengyu Huang, Jingyang Li, Zhi‐Dong Jiang, Y Liu, Wei Xiao, Jing Xu, Xiaofeng Zeng, Yu Zheng, Hejian Zou, R Becvar, Hanne Madsen, Klaus Søndergaard, Maritta Kilpeläinen, Marjukka Myllärniemi, C. Agard, Y Allanore, Arnaud Bourdin, V Cottin, B Crestani, Elizabeth Diot, S. Dominique, É. Hachulla, S. Jouneau, Sylvie Leroy, Hilario Nunès, Grégoire Prévôt, B. Wallaert, L Wemeau, Martin Aringer, Burkhard Bewig, Stefan Blaas, J. Distler, Jan Ehrchen, Ralf Ewert, Sven Gläser, J. Henes, Nicolas Hunzelmann, Ramona König, Ina Kötter, Michael Kreuter, Antje Prasse, Hendrik Schulze‐Koops, Petros P. Sfikakis, Panayiotis G. Vlachoyiannopoulos, György Losonczy, Digambar Behera, H J Gayathri Devi, Jugal K. Kadel, M Kawedia, Deepali Kumar, Umesh Kumar, Rahul Lokhande, Anand Malpani, Murali Mohan, A Nalawade, Ujjawal Parakh, Rajesh Swarnakar, Vineeta Shobha, Balamugesh Thangakunam, Zarir Udwadia, Michael Henry, K. O'Reilly, Alexandra Balbir‐Gurman, Mordechai R. Kramer, Irena Litinsky, Itzhak Rosner, Maurizio Cutolo, A Gabrielli
Abstract
OBJECTIVES: To investigate the course of interstitial lung disease (ILD) and the effects of nintedanib in patients with limited cutaneous systemic sclerosis (lcSSc). METHODS: In the SENSCIS trial, patients with SSc-ILD were randomized to receive nintedanib or placebo. Patients who completed the SENSCIS trial were eligible to enter SENSCIS-ON, in which all patients received open-label nintedanib. RESULTS: Among 277 patients with lcSSc treated in the SENSCIS trial, the rate (s.e.) of decline in forced vital capacity (FVC; ml/year) over 52 weeks was -74.5 (19.2) in the placebo group and -49.1 (19.8) in the nintedanib group (difference: 25.3 [95% CI -28.9, 79.6]). Among 249 patients with data at week 52, mean (s.e.) change in FVC at week 52 was -86.4 (21.1) ml in the placebo group and -39.1 (22.2) ml in the nintedanib group. Among 183 patients with lcSSc who participated in SENSCIS-ON and had data at week 52, mean (s.e.) change in FVC from baseline to week 52 of SENSCIS-ON was -41.5 (24.0) ml in patients who took placebo in the SENSCIS trial and initiated nintedanib in SENSCIS-ON and -45.1 (19.1) ml in patients who took nintedanib in the SENSCIS trial and continued it in SENSCIS-ON. CONCLUSION: Patients with lcSSc may develop progressive fibrosing ILD. By targeting pulmonary fibrosis, nintedanib slows decline in lung function in patients with lcSSc and ILD. TRIAL REGISTRATION: ClinicalTrials.gov (https://clinicaltrials.gov), NCT02597933 and NCT03313180.