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EULAR recommendations for the treatment of systemic sclerosis: 2023 update
Francesco Del Galdo1, Alain Lescoat2, Philip G Conaghan1
1Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, Leeds, UK; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK.
Annals of the Rheumatic Diseases
|January 28, 2025
Summary
Updated European Alliance of Associations for Rheumatology (EULAR) recommendations for systemic sclerosis (SSc) treatment now include targeted therapies for fibrotic manifestations and new guidelines for pulmonary arterial hypertension. These evidence-based guidelines aid rheumatology practitioners.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, vascular dysfunction, and immune dysregulation.
- The 2017 European Alliance of Associations for Rheumatology (EULAR) recommendations provided a framework for SSc management.
- Advances in understanding SSc pathogenesis and the emergence of novel therapies necessitated an update to existing guidelines.
Purpose of the Study:
- To comprehensively update the 2017 EULAR recommendations for the treatment of systemic sclerosis (SSc).
- To incorporate new evidence and emerging therapies into clinical practice guidelines.
- To provide updated guidance for managing key organ manifestations of SSc.
Main Methods:
- An international task force convened following EULAR standard operating procedures.
- A systematic literature review informed by nominal group technique and iterative voting rounds.
- Development of 22 recommendations across 8 clinical domains, including Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, scleroderma renal crisis, skin fibrosis, interstitial lung disease (ILD), gastrointestinal manifestations, and arthritis.
Main Results:
- The updated recommendations address 8 key clinical/organ domains of SSc.
- Novel recommendations include mycophenolate mofetil, nintedanib, rituximab, and tocilizumab for skin fibrosis and ILD.
- Guidelines now specify first-line and second-line interventions for various SSc manifestations, enhancing clinical utility.
- The research agenda prioritizes interventions for vascular, musculoskeletal, gastrointestinal issues, calcinosis, and digital ulcers.
Conclusions:
- These updated EULAR recommendations represent the first to include synthetic and biological targeted therapies for fibrotic SSc manifestations.
- First-line combination treatment for newly diagnosed pulmonary artery hypertension is now recommended.
- A prioritized research agenda for future SSc management strategies has been established.
