LONDON — Results from a longitudinal observational analysis of systemic sclerosis progression were presented on June 4, 2026. The study utilized data from the EUSTAR database, which tracks over 25,000 patients across more than 200 international centers.
Researchers stratified 3,214 patients from the database based on a modified disease activity index (mDAI) score. A score of less than 2.5 at baseline indicated inactive disease, while a score of 2.5 or higher signified active disease. Among the patients included in the analysis, 59.1 percent had inactive disease at baseline.
Patients who presented with active disease at baseline exhibited higher one-year cumulative incidence rates of organ and multi-system involvement compared to those with inactive disease. They also experienced shorter median times to several specific manifestations, including skin involvement, digital ulcers or gangrene, interstitial lung disease, and cardiac involvement. Digital ulcers can cause significant pain and physical impairment for patients with systemic sclerosis.
Further analysis showed that patients with active disease at baseline required more treatments at the monotherapy level than those with inactive disease. These treatments included immunosuppressive therapy, biologics, and antifibrotics. researcher 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."
Approved therapies and earlier vascular interventions have expanded treatment options for systemic sclerosis, prompting calls for more refined clinical assessments. The 2023 rheumatology guidelines update established a future research agenda, including interventions targeting vascular, musculoskeletal, and gastrointestinal manifestations, calcinosis, and digital ulcers.
A systematic literature review identified seven domains for standardized reporting in clinical trials on digital ulcers. These domains include a uniform definition and classification, clear inclusion and exclusion criteria for trial enrollment, and standardized outcome measures based on the healing of the primary ulcer and improvement in function. Recommended secondary outcomes for these trials include pain, function, quality of life, and patient-reported outcomes. The proposed guidelines recommend detailed reporting of background and concomitant therapies, local wound management protocols, and assessment timing with prespecified intervals and long-term follow-up for ulcer recurrence. The guidelines also state that trial design and analysis should consider seasonal and environmental factors, including climate impact.
No independent assessment was available for this report.