Systemic sclerosis (SSc) is a progressive disease with organ damage. Ethiopathology is associated with autoimmune inflammation, vasculopathy and fibrose.
Interstitial lung disease (ILD) is found in most patients with SSc and is the leading cause of death from SSc. Cyclophosphamide and mycophenolate mofetil are the most commonly indicated treatments for ILD associated with systemic scleroderma (SSc-ILD).
The Food and Drug Administration (FDA) and the European Medicines Agency (EMA) recently approved a new antifibrotic agent, nintedanib, for the treatment of SSc-ILD. The drug is indicated to slow the rate of decline in lung function in adults with SSc-ILD.