Evidence-based management of systemic sclerosis: Navigating recommendations and guidelines |
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| Institution: | 1. Center of Research of Immunopathology and Rare Diseases - Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences and University of Turin, Italy;2. SCU Nephrology and Dialysis Unit, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy;3. Rheumatology Unit, University Hospital, Padua, Italy;4. Apheresis Unit, University Hospital, Padua, Italy;1. Bristol Medical School, University of Bristol, Bristol, UK;2. Department of Pharmacy and Pharmacology, University of Bath, Bath, UK;3. Royal National Hospital for Rheumatic Diseases (part of Royal United Hospitals Bath NHS Foundation Trust), Upper Borough Walls, BA1, 1RL Bath, UK;1. Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France;2. INSERM, U995, F-59000 Lille, France;3. CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France;4. Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), F-59000 Lille, France;5. Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET);6. AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France;7. UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France;8. Service d''Immunologie Clinique, Hôpitaux universitaires de Strasbourg, UPR CNRS 3572, Strasbourg, France;9. Service de Médecine Interne et Maladies Multi-Organiques de l''Adulte, Hôpital Saint-Éloi, Centre Hospitalier Régional Universitaire de Montpellier, Montpellier, France;10. Hôpitaux Universitaires de Strasbourg, CHU Hautepierre, Service de Rhumatologie, Centre de Référence des Maladies Auto-Immunes Systémiques Rares, Université de Strasbourg, Fédération de Médecine Translationnelle de Strasbourg, INSERM UMR 1109, Strasbourg, France;11. Service de Médecine Interne, Centre Hospitalo-Universitaire de Rennes, Université de Rennes 1, Rennes, France;12. Service de Médecine Interne, Néphrologie et Médecine Vasculaire, Centre Hospitalier de Valenciennes, Valenciennes, France;13. Service de Dermatologie, Hôpital Tenon, AP-HP, UPMC, Paris, France;14. Service de Médecine Interne, Université Grenoble Alpes, Centre Hospitalier Universitaire (CHU) de Grenoble, Grenoble, France;15. Service de Médecine Interne, Hôpital Jean-Verdier, AP-HP, Université Paris-13, Bondy, France;p. Service de Médecine Interne et Rhumatologie, GH Diaconesses Croix Saint Simon, Paris, France;q. Service de Médecine Interne et Rhumatologie 3C/5D, Centre Hospitalier Universitaire Pierre Zobda-Quitman, Fort-de-France, Martinique;r. Service de Médecine Interne 2, Centre de Référence National pour le Lupus et le Syndrome des Antiphospholipides, institut E3M, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France;s. Sorbonne universités, UPMC université Paris 06, 75013 Paris, France;t. Service de Médecine interne, Hôtel-Dieu, CHU de Nantes, Université de Nantes, Nantes, France;u. CHU, Université de Toulouse, Faculté de Médecine, Service de Médecine Interne, Toulouse, France;v. INSERM, UMR 1027, Toulouse, France;1. Universitat Autònoma de Barcelona, Division of Rheumatology and Autoimmune Systemic Diseases, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain;2. Paris Descartes University, INSERM U1016, Sorbone Paris Cité, Rheumatology A Department, Cochin Hospital, Paris, France;3. Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), Florence, Italy;4. UO Reumatologia ed Immunologia Clinica Spedali Civili Brescia, Brescia, Italy;5. Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland;6. Fondazione IRCCS Ca'' Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy;7. Unita Operativa e Cattedra di Reumatologia. Policlinico Sant Matteo, Pavia, Italy;8. Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Hospital, University of Gothenburg. Gothenburg, Sweden;9. Rheumatology Unit-DETO, University of Bari. Bari. Italy;10. B. Shine Rheumatology Unit, Rambam Health Care Campus and Rappaport Faculty of Medicine, Haifa, Israel;11. Rutgers- Robert Wood Johnson Medical School Scleroderma Program, New Brunswick, United States |
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| Abstract: | ObjectivesSystemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed.MethodsThe updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted.ResultsThe updated EULAR/EUSTAR guidelines focus specifically on the management of SSc features and include data on newer therapeutic modalities and mention a research agenda. These recommendations are pharmacologic, with few guidelines regarding investigations and non-pharmacologic management. Recommendations from BSR/BHPR are similar to the organ manifestations mentioned in the EULAR/EUSTAR recommendations, and expand on several domains of treatment, including general measures, non-pharmacologic treatment, cardiac involvement, calcinosis, and musculoskeletal features. The guidelines usually agree with one another. Limitations include the lack of guidance for combination or second-line therapy, algorithmic suggestions, the absence of evidence-based recommendations regarding the treatment of specific complications (i.e., gastric antral ectasia and erectile dysfunction). Consensus for when to treat interstitial lung disease in SSc is lacking. There are differences between Europe and North American experts due to access and indications for certain therapies.ConclusionsCare gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc and future updates of these recommendations.Care gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc |
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| Keywords: | Systemic sclerosis Scleroderma Guidelines Recommendations Treatment Management Evidence based Best practices PAH ILD RP European Scleroderma Research Group British Society of Rheumatology |
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