The European Alliance of Associations for Rheumatology (EULAR) recently published updated recommendations on the treatment of systemic sclerosis (SSc).
SSc is a rare connective tissue disorder with both autoimmune and vascular features. The main hallmark is tissue and vascular fibrosis that can result in clinical heterogeneity of manifestations across the skin and internal organs. This heterogeneity makes the disease variable in both the symptoms it causes and how it should be treated. Of note, SSc has traditionally been called scleroderma, but strictly speaking this term refers only to skin involvement. Because non-skin manifestations have a significant impact, the term SSc is preferred in the EULAR recommendations.
Since its previous recommendations published in 2017, much new evidence – and new drugs – have become available for the management of SSc, thus EULAR’s treatment guidance has been reviewed and updated.
Researchers, healthcare professionals, and patients worked together to develop this new advice – including 27 people from 17 different countries. The group completed a literature review to collect up-to-date information, including findings from an online survey among people in EUSTAR – an international research network of over 200 specialist SSc centres. The new recommendations reflect the results of several clinical trials since the last update, with the demonstration of efficacy of both synthetic and biological therapies in some patient subsets.
The new recommendations, published in the 11 October 2024 issue of the Annals of the Rheumatic Diseases, include 22 individual recommendations across nine key categories. This is a significant increase from the 16 recommendations in 2017.
Several changes in the new recommendations are significant. These include the first set of synthetic and biological targeted therapies recommended for key fibrotic manifestations of SSc, as well as first-line combination treatment for newly-diagnosed pulmonary artery hypertension and prioritising a new research agenda in the coming years.
The new recommendations focus on the eight key clinical/organ domains of SSc including Raynaud’s phenomenon, digital ulcers, pulmonary arterial hypertension, scleroderma renal crisis, skin fibrosis, interstitial lung disease (ILD), gastrointestinal manifestations, and arthritis.
Most of the new recommendations are related to skin fibrosis and ILD. These include novel recommendations for the use of mycophenolate mofetil (MMF), nintedanib, rituximab, and tocilizumab for the treatment of these crucial disease manifestations.
The recommendations also include first-line and second-line interventions, providing increased utility for rheumatology practitioners.
Important additions to the future research agenda included consideration of novel interventions for the management of vascular, musculoskeletal, and gastrointestinal manifestations and calcinosis, as well as for the local management of digital ulcers.
A one-size-fits-all strategy cannot be implemented in SSc, where disease duration, comorbidities, patient preference, local availability, and cost of medication all need to be taken into account for informed decision-making, the EULAR document maintains.
In summary, the new recommendations note that the big advances made in SSc vasculopathy management emphasise the treatment continuum for the use of various vasodilators and anti-remodelling drugs from Raynaud’s to digital ulcers and pulmonary arterial hypertension. Similarly, a therapeutic continuum in the interventions for skin and lung fibrosis is also apparent. These latter two domains are the ones with the most important updated information, resulting in recommendations for the use of MMF and/or rituximab, and tocilizumab for both skin and lung, and nintedanib to be used alone or in combination with MMF for the treatment of SSc-ILD.
EULAR is confident the new recommendations provide state-of-the-art guidance for physicians globally to help manage SSc patients across the spectrum of disease.
Source
Del Galdo F, et al. EULAR recommendations for the treatment of systemic sclerosis: 2023 update. Ann Rheum Dis. 2024 Oct 17:ard-2024-226430
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