Systemic Sclerosis (SSc)

In this episode, we explore Systemic Sclerosis (SSc), examining its defining pathogenic triad of vasculopathy, autoimmunity, and fibrosis. We discuss the 2013 ACR/EULAR classification criteria, which improved diagnosis by weighing early signs like Raynaud’s phenomenon, puffy fingers, and specific autoantibodies alongside skin thickening. The episode covers critical organ risks—specifically Interstitial Lung Disease (ILD) and Pulmonary Arterial Hypertension (PAH) as leading causes of mortality—and the role of biomarkers like anti-topoisomerase I and anti-RNA polymerase III in predicting complications such as cardiac fibrosis and Scleroderma Renal Crisis. Finally, we review the therapeutic landscape, moving from established treatments like ACE inhibitors and nintedanib to emerging frontiers like CAR-T cell therapy and autologous hematopoietic stem cell transplantation.