Systemic Sclerosis

Systemic sclerosis (SSc), also known as scleroderma, is a chronic orphan disease of an unknown etiology, characterized by autoimmunity mediated through specific autoantibodies and auto-reactive T cells. It is hallmarked by dermal and visceral fibrosis, alongside a concurrent vasculopathy terrain reminiscent of Raynaud’s phenomenon, pulmonary arterial hypertension, and less commonly, scleroderma renal crisis. Despite its relatively low prevalence, the disease exerts a substantial burden. Recent milestones, including the 2013 ACR/EULAR classification criteria, coupled with a diverse array of reviews and research advancements, have notably enhanced our insights into its pathogenesis and clinical manifestations, expanding our therapeutic armamentarium. The paramount significance of early and precise diagnosis is underscored, harmonized with ongoing endeavors to stratify patient risks. This serves as a crucial element in predicting both organ involvement and the course of disease progression. Considering the heterogeneous expression of the disease, a comprehensive evaluation of the patient becomes imperative. This encompasses the selection of a therapeutic approach that not only attains therapeutic efficacy but also minimizes associated adverse effects. Crafting the most effective therapeutic strategy demands a holistic approach, meticulously calibrating therapeutic impact against potential side effects, all the while aligning interventions with the specific care goals of the patient.