Systemic sclerosis (SSc) is a rare progressive autoimmune disorder characterized by skin fibrosis, systemic inflammation, and vasculopathy that manifests as Raynaud’s disease and, often, painful digital ulcers. Risks of pulmonary arterial hypertension(PAH), interstitial lung disease (ILD), and renal crisis are high in SSc patients. The only disease-modifying therapy (DMT) approved to treat SSc is Ofev, targeted for a subpopulation of SSc patients suffering from ILD. Boehringer Ingelheim’s Ofev awaits approval in the European Union (EU) for SSc-ILD. Ofev has the potential to advance the treatment of SSc-ILD, which partially underlies the high mortality rate in SSc.ILD and skin fibrosis are treated with systemic immunosuppressants; symptoms of PAH and vasculopathy are treated with varying combinations of vasodilators; and renal crisis is treated acutely with angiotensin-converting exzyme (ACE) inhibitors. SSc is a debilitating condition, and treatments are only marginally effective, leaving tremendous need for effective DMTs and antifibrotics.