Early Systemic Sclerosis: What’s in a Name

Abstract Purpose of the review This review aims to clarify the different definitions and contexts of the term "early" in systemic sclerosis (SSc) and its implications in clinical practice and research. The term "early" has been used inconsistently, leading to confusion in diagnosing, managing, and conducting research on SSc. Recent findings Recent studies have explored the evolution of patients from very early SSc stages to established disease. The development of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria has marked a significant step forward. However, studies have shown varying progression rates among patients meeting different "early" criteria, with a subset remaining in mild or indolent stages for extended periods. Significant gaps remain in predicting disease progression, prompting the need for more refined and personalized criteria. Summary Systemic sclerosis is a complex disease with high morbidity and mortality. The inconsistent use of the term "early" has led to challenges in diagnosis, treatment, and research. While the VEDOSS criteria have provided a framework for identifying very early SSc, further refinement is needed to improve disease progression predictions. A more personalized approach to patient monitoring and the application of adjusted VEDOSS criteria may enhance early intervention strategies, ultimately aiming to prevent disease progression in SSc.