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

PURPOSE OF REVIEW: 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 FINDINGS: 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. SUMMARY: 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.