The European Alliance of Associations for Rheumatology released updated recommendations for the treatment of systemic sclerosis, incorporating new evidence and therapies.
The 2023 update, published in Annals of the Rheumatic Diseases, featured 22 recommendations covering 8 clinical domains, an increase from 16 recommendations in 2017.
In the new guidelines, a task force provided updated recommendations for treating skin fibrosis and interstitial lung disease (ILD) with therapies such as mycophenolate mofetil, nintedanib, rituximab, and tocilizumab. In pulmonary arterial hypertension (PAH), first-line combination therapy with phosphodiesterase-5 inhibitors and endothelin receptor antagonists was recommended for newly diagnosed cases. Intravenous epoprostenol remained recommended for advanced PAH, and anticoagulants (warfarin) were explicitly not recommended for systemic sclerosis (SSc)-PAH.
For SSc-related ILD, the task force suggested mycophenolate mofetil, cyclophosphamide, or rituximab as first-line options, with nintedanib recommended alone or in combination with mycophenolate mofetil. Tocilizumab was also recommended for consideration in SSc-ILD.
In skin fibrosis, methotrexate, mycophenolate mofetil, and rituximab were advised, while tocilizumab was suggested for consideration in early, inflammatory diffuse cutaneous SSc.
The task force emphasized the vascular therapeutic continuum across Raynaud's phenomenon, digital ulcers, and PAH, noting that these conditions share common disease mechanisms. Calcium channel blockers remained the first-line therapy for Raynaud's, with phosphodiesterase-5 inhibitors as an alternative. For digital ulcers, phosphodiesterase-5 inhibitors and/or intravenous iloprost were recommended, whereas bosentan was advised for reducing the number of new digital ulcers.
The recommendations were developed by an international task force of 27 members from 17 countries, following The European Alliance of Associations for Rheumatology (EULAR) standardized operating procedures. A systematic literature review from January 2015 to March 2023 informed the new guidance. The task force agreed on 22 recommendations with varying levels of evidence (LoE) and strengths of recommendation (SoR). For instance, the recommendation for combination PDE-5 inhibitors and endothelin receptor antagonists as first-line treatment for SSc-PAH had LoE 1a and SoR A, while tocilizumab for early inflammatory diffuse cutaneous SSc had LoE 1b and SoR C.
The task force also highlighted areas for further research, particularly on novel interventions for vascular, musculoskeletal, and gastrointestinal manifestations as well as the management of calcinosis.
These updated recommendations may guide clinicians on the use of newer therapies and combination treatments for key SSc manifestations, reflecting the latest evidence on SSc pathophysiology and treatment approaches, particularly for fibrotic and vascular complications.
Conflict of interest disclosures can be found in the guidelines.