2023 UPDATE OF EULAR RECOMMENDATIONS FOR THE TREATMENT OF SYSTEMIC SCLEROSIS

F. Del Galdo, A. Lescoat, P. G. Conaghan, L. P. Ananyeva, A. Balbir-Gurman, E. Bertoldo, V. Boyadzhieva, I. Castellví, J. Colic, C. P. Denton, O. Distler, K. El Aoufy, J. Emmel, S. Farrington, A. Gabrielli, I. Galetti, A. M. Hoffmann-Vold, O. Kowal-Bielecka, M. Matucci Cerinic, U. Müller-Ladner, P. Ostojic, S. Rednic, T. Santiago, Y. A. Suliman, A. Tarasova, M. Vonk, Y. AllanoreUniversity of Leeds, Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom Leeds, Biomedical Research Centre, Leeds, United Kingdom Univ Rhennes, CHU Rennes, Rennes, France V.A.Nasonova, Reseach Institute of Rheumatology, Moscow, Russian Federation BRambam Health Care Campus,. Shine Rheumatology Institute, Haifa, Israel University of Verona, Rheumatology, Verona, Italy University Hospital “St. Ivan Rilski”, Department of Rheumatology, Sofia, Bulgaria Hospital Universitari de la Santa Creu i Sant Pau, Department of Rheumatology and Systemic Autoimmune Diseases., Barcelona, Spain Institute of Rheumatology, Department of Rheumatology Unit, Belgrade, Serbia University College London, Rheumatology, London, United Kingdom University Hospital, Rheumatology, Zurich, Switzerland University of Florence, Department of Experimental and Clinical Medicine, Florence, Italy Leeds Teaching Hospital Trust, Library, Leeds, United Kingdom FESCA, SRUK, London, United Kingdom Universita’ di Ancona, Rheumatology, Ancona, Italy FESCA, GILS, Milan, Italy Oslo University Hospital, Rheumatology, Oslo, Norway Medical University of Bialystok, Department of Rheumatology and Internal Medicine, Bialystok, Poland San Raffaele, Rheumatology, Milan, Italy Bad Nauheim, Justus Liebig University Giessen, Bad Nauheim, Germany Belgrade, Rheumatology, Belgrade, Serbia Cluj-Napoca, Department Rheumatology, Cluj-Napoca, Romania Centro Hospitalar e Universitário de Coimbra, Rheumatology, Coimbra, Portugal Assiut university Hospital, Rheumatology and Rehbilitation departement, Asyut, Egypt Moscow, Rheumatology, Moscow, Russian Federation Radboud University Nijmegen Medical Center, Department of Rheumatology, Nijmegen, Netherlands Cochin University Hospital, Rheumatology, Paris, France  Background EULAR recommendations for treatment for Systemic Sclerosis (SSc) were last updated in 2017, based on literature up to the end of 2014. The treatment recommendations are among the most cited publication in the field, reflecting their utility both in clinical practice and academic research. In the past 8 years, several important advances have been achieved by the scientific community, with new treatments becoming available and accepted by regulatory authorities. Objectives To update the 2017 EULAR recommendations for treatment of SSc, including answers to the 2017 research agenda, new therapeutic questions, and the wealth of newly available evidence. Methods Update of previous recommendations was endorsed by EULAR and performed according to the validated methodology. The task force was composed according to recent EULAR guidelines for inclusivity of underrepresented areas of Europe and it consisted of 27 members, including a EULAR endorsed methodologist, 4 fellows, a librarian, one Allied Health Professional and two patient representatives, identified by the Federation of European Scleroderma Associations (FESCA). All centres from the EULAR Scleroderma Trials and Research group (EUSTAR) were invited to review previous questions for Systematic Literature Review (SLR) and propose new ones according to a standardised Delphi approach. A Nominal Group Technique (NGT) exercise was implemented in two rounds for the definition of questions with an 80% agreement threshold. The SLR was conducted with an end date of June 2022, and manually updated to December 2022. Particular attention was dedicated to the 2017 research agenda and new available evidence with matters for detailed discussion and new research agenda also agreed during the NGT. Results Sixty-seven clinical questions addressing 24 different interventions were prioritised by the task force for the SLR. The results of the SLR were categorised by level of evidence and grade of recommendations and discussed during the NGT meeting. The procedure resulted in 21 recommendations (instead of 16 in 2017) in 8 clinical domains related to SSc symptoms and organ involvements, including Raynaud’s phenomenon, digital ulcers, pulmonary arterial hypertension, scleroderma renal crisis, skin fibrosis, interstitial lung disease, gastrointestinal manifestations and arthritis. Most of the new recommendations were in the field of skin fibrosis and interstitial lung disease, consistent with the research agenda set in 2017. These included recommendations for the use of mycophenolate mofetil, nintedanib, rituximab and tocilizumab for the treatment of these crucial disease manifestations. None of these therapies were present in the 2017 recommendations. Important additions to the future research agenda included targeted therapies for the management of musculoskeletal and gastrointestinal manifestations, calcinosis and the local management of digital ulcers. Conclusion The 2023 updates of EULAR recommendations include the first set of synthetic and biological targeted therapies recommended as disease modifying agents for key fibrotic manifestations of systemic sclerosis. While a disease modifying agent or strategy for a comprehensive approach appropriate for all SSc patients is still missing, numerous advances have been achieved since 2017. New, strong evidence is now available to clinicians to better manage patients with this life-threatening condition. Reference [1]Kowal-Bielecka O, Fransen J, Avouac J, et al. Ann Rheum Dis 2017;76:1327-1339 Acknowledgements: NIL. Disclosure of Interests Francesco Del Galdo Speakers bureau: Astra-Zeneca, Janssen, Consultant of: Astra Zeneca, Boehringer-Ingelheim, Capella, Chemomab, Janssen, Mitsubishi-Tanabe, Grant/research support from: Abbvie, AstraZeneca, Boheringer-Ingelheim, Capella, Chemomab, Kymab, Janssen, Mitsubishi-Tanabe, Alain LESCOAT: None declared, Philip G Conaghan: None declared, Lidia P. Ananyeva Consultant of: Boehringer Ingelheim and Biocad, Alexandra Balbir-Gurman: None declared, Eugenia Bertoldo: None declared, Vladimira Boyadzhieva: None declared, Ivan Castellví Speakers bureau: Boehringer Ingelheim, Kern, Galápagos, Topandur, & Janssen- Cilag, Consultant of: Boehringer Ingelheim, Kern, Galápagos, Topandur, & Janssen- Cilag, Jelena Colic: None declared, Christopher P Denton Speakers bureau: GlaxoSmithKline, Galapagos, Boehringer. Ingelheim, Roche, CSL Behring, Corbus, Horizon, Capella Bioscience and Arxx Therapeuti, Consultant of: GlaxoSmithKline, Galapagos, Boehringer. Ingelheim, Roche, CSL Behring, Corbus, Horizon, Capella Bioscience and Arxx Therapeuti, Grant/research support from: GlaxoSmithKline, Galapagos, Boehringer. Ingelheim, Roche, CSL Behring, Corbus, Horizon, Capella Bioscience and Arxx Therapeuti, Oliver Distler Speakers bureau: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur, Consultant of: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur, Khadija El Aoufy: None declared, Jenny Emmel: None declared, Sue Farrington: None declared, Armando Gabrielli: None declared, Ilaria Galetti: None declared, Anna-Maria Hoffmann-Vold Speakers bureau: Boehringer Ingelheim, Jannsen, Medscape, Merck Sharp & Dohme and Roche, Consultant of: ARXX, Boehringer Ingelheim, Genentech, Jannsen, Medscape, Merck Sharp & Dohme and Roche, Grant/research support from: Boehringer Ingelheim, Jannsen, Otylia Kowal-Bielecka Speakers bureau: Abbvie, Boehringer Ingelheim, CSL Behring, Janssen-Cilag, Medac, MSD, Novartis, Pfizer, Sandoz, Consultant of: Abbvie, Boehringer Ingelheim, CSL Behring, Janssen-Cilag, Medac, MSD, Novartis, Pfizer, Sandoz, Grant/research support from: Abbvie, Boehringer Ingelheim, CSL Behring, Janssen-Cilag, Medac, MSD, Novartis, Pfizer, Sandoz, Marco Matucci Cerinic Speakers bureau: Bayer,Biogen, Boehringer Ingelheim, Corbus, CSL Behring, Galapagos, Inventiva,Lilly, MSD, Sandoz, Consultant of: Bayer,Biogen, Boehringer Ingelheim, Corbus, CSL Behring, Galapagos, Inventiva,Lilly, MSD, Sandoz, Grant/research support from: Bayer,Biogen, Boehringer Ingelheim, Corbus, CSL Behring, Galapagos, Inventiva,Lilly, MSD, Sandoz, Ulf Müller-Ladner Speakers bureau: BMS, Galapagos, Medac, Novartis, Janssen, Lilly, Boehringer, Consultant of: BMS, Galapagos, Medac, Novartis, Janssen, Lilly, Boehringer, Predrag Ostojic Speakers bureau: Abbvie, Eli Lilly, Boehringer Ingelheim, Novartis, MSD, Pfizer, Sandoz, Berlin Chemie Menarini, Amicus, EL Pharma, Consultant of: Abbvie, Eli Lilly, Boehringer Ingelheim, Novartis, MSD, Pfizer, Sandoz, Berlin Chemie Menarini, Amicus, EL Pharma, Simona Rednic Speakers bureau: Abbvie, Amgen, AstraZeneca, Boehringer Ingelheim, Ewopharma, Janssen, Novartis, Pfizer, Sanofi, Sandoz, Consultant of: Abbvie, Amgen, AstraZeneca, Boehringer Ingelheim, Ewopharma, Janssen, Novartis, Pfizer, Sanofi, Sandoz, Grant/research support from: Abbvie, Amgen, AstraZeneca, Boehringer Ingelheim, Ewopharma, Janssen, Novartis, Pfizer, Sanofi, Sandoz, Tânia Santiago: None declared, Yossra A. Suliman: None declared, Anna Tarasova: None declared, Madelon Vonk Speakers bureau: Boehringer Ingelheim, Ferrer, Galapagos, GSK, Janssen, MSD and Novartis, Consultant of: Boehringer Ingelheim, Ferrer, Galapagos, GSK, Janssen, MSD and Novartis, Yannick Allanore Consultant of: AbbVie, AstraZeneca, Bayer, Boehringer-Ingelheim, Mylan, Janssen, Medsenic, Prometheus, Sanofi, Roche, Grant/research support from: Alpine Immunosciences, Medsenic, OSE Immunotherapeutics. Keywords: Systematic review, Clinical trials, Disease-modifying drugs (DMARDs) DOI: 10.1136/annrheumdis-2023-eular.1383Citation: , volume 82, supplement 1, year 2023, page 154Session: Update on the treatment of scleroderma lung disease (Oral Presentations)