EULAR recommendations for the treatment of systemic sclerosis: 2023 update

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, vascular dysfunction, and immune dysregulation.
• Existing treatment guidelines require updates to reflect advancements in therapeutic options and understanding of SSc pathophysiology.

Purpose of the Study:

• To revise and update the 2017 European Alliance of Associations for Rheumatology (EULAR) recommendations for managing systemic sclerosis (SSc).
• To integrate new evidence and emerging therapies into clinical practice guidelines for SSc treatment.

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:

• New recommendations focus on skin fibrosis and interstitial lung disease (ILD), incorporating mycophenolate mofetil, nintedanib, rituximab, and tocilizumab.
• Guidelines now include first-line and second-line interventions for improved clinical utility.
• The updated agenda prioritizes research into novel treatments for vascular, musculoskeletal, gastrointestinal manifestations, calcinosis, and digital ulcers.

Conclusions:

• These updated EULAR recommendations introduce targeted synthetic and biological 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.