Study shows early disease activity predicts systemic sclerosis progression

In an oral abstract presentation on Thursday 4th June, results were shared from a longitudinal observational analysis of the EUSTAR database – a large multicenter online database that prospectively follows more than 25,000 patients across over 200 international centers. The aim was to characterise organ involvement and disease progression – and time to these events – stratified by having a modified disease activity index (mDAI) score of less than 2.5 at baseline (inactive), versus those with 2.5 or higher (active). Of the 3,214 patients included, 59.1% had inactive disease. Those with active disease at baseline showed higher cumulative incidence rates of organ and multi-system involvement at 1 year, underscoring the relevance of disease-activity monitoring. This was particularly true for skin involvement, digital ulcers/gangrene, interstitial lung disease, and cardiac involvement – with shorter median times to these events for active versus inactive disease. Patients with active disease also required more immunosuppressive therapy, biologics, and antifibrotics as monotherapy compared to those with inactive disease. 

Presenting the work at the Congress in London, Tânia Santiago said "This study provides novel insights into the natural history of systemic sclerosis, as stratified by the mDAI, underscoring the importance of early disease activity assessment". 

A poster from Corrado Campochiaro and colleagues considered points-to-consider for reporting outcomes measures in interventional studies on digital ulcers – an initiative from a committee of the World Scleroderma Foundation. Digital ulcers are one of the most painful and disabling aspects of systemic sclerosis, but there is a lack of consistency in their definition and reporting. The work – based on a systematic literature review – identified seven domains as essential for standardised reporting in clinical trials. These include uniform definition and classification, clear inclusion and exclusion criteria for trial enrolment, and standardised outcome measures based around healing of the cardinal ulcer and improvement in function, with secondary outcomes including pain, function, quality of life, and patient-reported outcomes. The points-to-consider also emphasise the need for detailed reporting of background and concomitant therapies, local wound management protocols, and the timing and frequency of assessments – with prespecified intervals and long-term follow-up for ulcer recurrence. Finally, trial design and analysis should consider seasonal and environmental factors such as the impact of climate. The authors believe that adoption could help to enhance the quality, consistency, and interpretability of interventional studies, as well as facilitating more robust meta-analyses, improving comparability across trials, and ultimately support the development of effective, patient-centred therapies. 

As noted in the 2023 update of the EULAR guidelines, the future research agenda includes 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. In light of this, the new findings showcased at EULAR 2026 are important. The field is evolving, with approved drugs and earlier vascular interventions – and this calls for more refined assessment.