Abstract / Summary
Systemic sclerosis (SSc) is classed as a rare or ‘orphan disease’ with significant clinical and molecular heterogeneity causing multi-organ fibrosis, vasculopathy and immune dysregulation, and carries a high morbidity and mortality burden. Recent guidelines of the international rheumatological, respiratory and cardiovascular societies provide state of the art updates rooted in evidence-based management. In this narrative review, we summarise current best practice of this complex disease drawing from guidelines and insights from translational, clinical and randomised controlled studies. We explore the role of multi-omics and technologies such as artificial intelligence in patient stratification and intelligent trial design. New treatment targets including groundbreaking cellular therapies which hold significant potential to add to our armamentarium of SSc tools, are also discussed. Despite recent strides in progress, there remains significant unmet need in understanding pathogenic mechanisms in SSc and addressing the non-lethal burden of disease on the road towards achieving true disease modification targeted to the right patient population at the right time. This review ultimately aims to provide an updated roadmap for managing a ‘hard condition’ by application of latest advances in precision-based medicine.