Schematic representation of the eight clinical domains covered by the 2023 recommendations. Note that severe prognosis is not represented. The different shades of green boxes labelled A–D represent the Strength of the Recommendation (SoR) as shown in the relative column of table 1. Dotted lines connect same drug or drug class across distinct clinical domains. CCB, calcium channel blocker; CYC, cyclophosphamide; ERAs, endothelin receptor antagonists; MMF, mycophenolate mofetil; MTX, methotrexate; PDE5i, phosphodiesterase five inhibitors; PPI, proton pump inhibitors; RITUX, rituximab; TCZ, tocilizumab. Credit: Annals of the Rheumatic Diseases (2024). DOI: 10.1136/ard-2024-226430

Systemic sclerosis (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 sometimes also 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.

The last version of the recommendations was published in 2017, but since then there have been new treatments developed, and new evidence collected. To address this, EULAR has reviewed and updated the recommendations. Researchers, health care 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. This was based on findings from an online survey among people in EUSTAR—an international research network of over 200 specialist SSc centers. The evidence generated was reviewed, grouped according to clinical domain and presented to the task force who voted on and discussed the impact of the new data on the existing recommendations

The new recommendations, published in the Annals of the Rheumatic Diseases, includes 22 individual recommendations, split across nine key categories. This is an increase from the 16 recommendations in 2017. The new recommendations focus on the eight key manifestations of SSc, as well as consideration of people with poor prognosis.

The first group of recommendations is aimed at people with Raynaud’s phenomenon, where blood flow is reduced in the fingers and toes. This is followed by specific recommendations for people whose SSc causes ulcers on their fingers, then those with pulmonary arterial hypertension (PAH), and people with scleroderma renal crisis—where a sudden increase in blood pressure is associated with rapidly declining kidney function. Other manifestations include skin fibrosis or thickening, interstitial lung disease (ILD), gastrointestinal manifestations, and arthritis.

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. A one-size-fits-all strategy cannot be implemented in SSc, where disease duration, comorbidities, patient preference, local availability, and cost of medication al need to be taken into account for informed decision-making. EULAR is confident the new recommendations provide state-of-the-art guidance for physicians globally to help them manage their SSc patients across the spectrum of disease.