OP0225 CHANGES IN TREATMENT PATTERNS AND THEIR INFLUENCE ON THE OUTCOME OF SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE (SSc-ILD) PATIENTS: AN EUSTAR ANALYSIS (2024)

OP0225 CHANGES IN TREATMENT PATTERNS AND THEIR INFLUENCE ON THE OUTCOME OF SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE (SSc-ILD) PATIENTS: AN EUSTAR ANALYSIS (1)

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Scientific Abstracts

Oral Abstract Presentations

Clinical Abstract Sessions: Treatment options in Systemic sclerosis

OP0225 CHANGES IN TREATMENT PATTERNS AND THEIR INFLUENCE ON THE OUTCOME OF SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE (SSc-ILD) PATIENTS: AN EUSTAR ANALYSIS

  1. C. Campochiaro1,
  2. M. E. Truchetet2,
  3. M. Vonk3,
  4. G. Cuomo4,
  5. L. P. Ananyeva5,
  6. E. Hachulla6,
  7. V. Smith7,
  8. A. M. Gheorghiu8,
  9. R. Bečvář9,
  10. P. Carreira10,
  11. N. Hunzelmann11,
  12. D. Furst12,
  13. V. Ortiz-Santamaria13,
  14. F. Del Galdo14,
  15. M. Matucci-Cerinic1,
  16. A. M. Hoffmann-Vold15,16
  1. 1IRCCS San Raffaele Hospital, Unit of Immunology, Rheumatology, Allergy and Rare Disease. Vita-Salute San Raffaele University, Milan, Italy
  2. 2Bordeaux University Hospital, Bordeaux, France
  3. 3Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands
  4. 4Department of Precision Medicine, “Luigi Vanvitelli” University of Campania, Naples, Italy
  5. 5V.A. Nasonova Research Institute Of Rheumatology Russian Federation, Moscow, Russian Federation
  6. 6University of Lille, Lille, France
  7. 7University of Ghent, Ghent, Belgium
  8. 8Department of Internal Medicine and Rheumatology, Ion Cantacuzino Clinical Hospital, Bucharest, Romania
  9. 9Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic
  10. 10Hospital Universitario 12 de Octubre, Madrid
  11. 11University of Kšln, Kšln, France
  12. 12University of California Los Angeles, Los Angeles, United States of America
  13. 13University of Granollers, Granollers, Spain
  14. 14University of Leeds - Leeds Institute of Rheumatic and Muskuloskeletal Medicine, Leeds, United Kingdom
  15. 15Oslo University Hospital, Department of Rheumatology, Oslo, Norway
  16. 16Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland

Abstract

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 <0.001). The treatment pattern after progression also differed among periods with increase in combination and switch in ISTs (Figure 1A). We identified improved progression free survival from period 2 to 3 and 4 with 46.3%, 64.9% and 55.0%; p = 0.007 (Figure 1B). In the ATA positive SSc-ILD patients subanalysis (136, 311, 185 and 154 in period 1-4), excluding period 1 we identified significantly fewer progressive events on IST with 73/318 (23.0%), 84/563 (14.9%) and 83/654 (12.7%) in period 2 compared to 3 and 4 (p = <0.001). Progression-free survival improved from 46.9%, to 67.1% and 57.6% in period 2, 3 and 4; p = 0.045 (Figure 1C).

Table 1. Clinical and treatment features of SSc-ILD patients at first-time assessment across the 4 periods. Data are presented and numbers and (%).

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Figure 1.

Panel A. ISTs and treatment regimens across the 4 periods. Panel B Progression-free survival of SSc-ILD patients across the 3 periods in the whole cohort, and Panel C in the enriched cohort.

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.

  • Real-world evidence
  • Lungs
  • Registries

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    OP0225 CHANGES IN TREATMENT PATTERNS AND THEIR INFLUENCE ON THE OUTCOME OF SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE (SSc-ILD) PATIENTS: AN EUSTAR ANALYSIS (2024)

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