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Background: The treatment armamentarium with immunosuppressive treatments (ISTs) for interstitial lung disease (ILD) in systemic sclerosis (SSc) has greatly expanded over the past decades. The implementation in clinical practice and the impact of IST on ILD progression is to date unclear. To understand this is of importance for the clinical management of patients and inclusion of patients into clinical trials. Objectives: Assess treatment regimens in SSc-ILD over time including initiation, switch and stop of ISTs and its impact on ILD progression and progression free survival. Methods: SSc patients registered in the EUSTAR database with presence of ILD on imaging, available FVC%, DLCO% predicted and ISTs at baseline and at 12 ± 3 months were included. We segregated treatment regimens into four periods based on patient's first assessment: 1) ≤ 2006; 2) 2007-2011; 3) 2012-2016; 4) ≥ 2017 and assessed clinical characteristics and type of IST. Next, we evaluated the impact of ISTs across a 3-year follow-up period on the two outcomes, (I) progressive ILD events and (II) progression free survival. Absolute decline of FVC≥5% or DLCO≥10% over 12 ± 3 months was defined as a progressive event and progression free survival as survival in the absence of progression within 36 months. Next, we assessed the impact of ISTs on these two endpoints in patients with positive anti-topoisomerase I (ATA) to enrich for progressors and evaluated treatment changes after periods of progression. Non-parametric tests were used for multiple group comparisons. Results: In total, 1409 SSc–ILD patients were included with 236 (16.7%) in period 1; 558 (39.6%) in period 2; 338 (23.9%) in period 3 and 277 (19.7%) in period 4. The clinical characteristics were comparable across the periods (Table 1). The use of ISTs increased significantly from 13.6 % in period 1 to 57.4% in period 4 with a significant change of type of IST, and frequency of switches and combination in ISTs and time to treatment stop (Figure 1A). When assessing the impact of ISTs over the follow-up of mean 3 years (excluding period 1), we identified significantly fewer progressive events; with 115/540 (21.3%), 146/1061 (13.8%) and 160/1320 (12.1%) in period 2, 3 and 4, respectively (p Table 1. Clinical and treatment features of SSc-ILD patients at first-time assessment across the 4 periods. Data are presented and numbers and (%). Conclusion: The majority of SSc-ILD patients are currently started on IST and over time there has been a significant change in treatments regimens with novel therapies being implemented in clinical practice especially in case of patients' functional worsening. While this has reduced the number of observed progressive ILD events, progression-free survival is still unsatisfying with 45% surviving at 3 years. This represents a clear need for the development of novel treatment options and treatment regimens. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: Corrado Campochiaro Boehringer Ingelheim, Janssen, Novartis, Marie-Elise Truchetet AbbVie/Abbott, Boehringer-Ingelheim, Gilead, Merck/MSD, UCB, Madelon Vonk Boehringer Ingelheim, Corbus, Ferrer, Galapagos, Janssen, MSD, Giovanna Cuomo: None declared, Lidia P. Ananyeva: None declared, Eric Hachulla Bayer, CSL Behring, GlaxoSmithKlein(GSK), johnson&Johnson, Novartis, Otsuka, Roche-Chugai, sanofi-genzyme, Sobi, Vanessa Smith Boehringer Ingelheim, Support for travel, Galapagos, Janssen-Cilag,, Ana Maria Gheorghiu AbbVie/Abbott, Boehringer-Ingelheim, Ewopharma, Sandoz,, Radim Bečvář: None declared, Patricia Carreira: None declared, Nicolas Hunzelmann: None declared, Daniel Furst Amgen, Corbus, CSL Behring, Galapagos, Gilead, GSK, Horizon, Novartis, Pfizer, Roche, Vera Ortiz-Santamaria: None declared, Francesco Del Galdo AbbVie/Abbott, Arxx, AstraZeneca, Boehringer-Ingelheim, Capella, Chemomab, GlaxoSmithKlein(GSK), Janssen, Mitsubishi-Tanabe, Marco Matucci-Cerinic accelerong, actelion, bayer, biogen, Boehringer-Ingelheim, Chemomab, corbus, CSL Behring, Eli Lilly, galapagos, Inventiva, Janssen, Merck/MSD, Mitsubishi, Pfizer, regeneron, Roche, samsung, Anna-Maria Hoffmann-Vold Boehringer Ingelheim, Boehringer Ingelheim, Janssen, Medscape, Merck Sharp & Dohme, Novartis and Roche, ARXX, BMS, Boehringer Ingelheim, Genentech, Janssen, Medscape, Merck Sharp & Dohme and Roche, Boehringer Ingelheim, Janssen.
Campochiaro et al. (Sat,) studied this question.
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