Modern drug therapy for systemic sclerosis associated interstitial lung disease

Systemic Sclerosis with Interstitial Lung Disease (SSc-ILD) is associated with an increased risk of morbidity and mortality. Because of a lack of approved medications that can effectively influence SSc-ILD disease course, there is a need for new therapeutic options. Treatment with immunomodulatory therapies as well as with autologous stem cell transplant is being further investigated in current clinical studies. Recently, a phase III study demonstrated the positive effect of the antifibrotic agent nintedanib on the loss of lung volume and thus disease progression in patients with SSc-ILD. Due to its synergistic mechanism of action, combination therapy with nintedanib and mycophenolate could be a complementary treatment approach for SSc-ILD in the future.