Systemic sclerosis in 2026: emerging paradigms from the World Scleroderma Congress

To summarize recent advances in systemic sclerosis (SSc) highlighted at the 2026 World Scleroderma Congress, focusing on early disease, immunovascular mechanisms, and emerging therapeutic strategies.

Recent data support a paradigm shift in SSc, demonstrating that disease onset occurs during a very early phase (VEDOSS) characterized by early immune activation and microvascular dysfunction. Type I interferon-driven pathways are increasingly implicated in vascular and myocardial involvement, reinforcing the concept of a shared immunovascular axis across organ systems. Advances in multimodal imaging and circulating biomarkers enable detection of subclinical organ disease, while multi-omic and data-driven approaches are redefining disease subsets beyond traditional classifications. In parallel, novel therapeutic strategies - including targeted immune modulation, autologous haematopoietic stem cell transplantation (AHSCT), and emerging CAR-T approaches - are driving a transition toward earlier, mechanism-based intervention.

Systemic sclerosis is increasingly understood as a dynamic continuum of autoimmune/inflammatory and fibrotic mechanisms that can be identified through imaging, biohumoral, and functional insights, thus enabling a very early/early diagnosis, a risk stratification, and a precision-based therapeutic approach.