Introduction: Interstitial lung disease (ILD) is a leading cause of death in patients with systemic sclerosis (SSc). Nonspecific immunosuppressants have been the first-line treatment for SSc-associated ILD (SSc-ILD). Nintedanib, an oral triple kinase inhibitor targeting profibrotic pathways, has been employed for the treatment of idiopathic pulmonary fibrosis and has recently received marketing approval in the United States and Japan, based on the results of a placebo-controlled randomized controlled trial. In this clinical trial, nintedanib delayed the progression of SSc-ILD compared with placebo.
Areas covered: This review covers current pharmacotherapies for SSc-ILD, drug profiles of nintedanib, and efficacy and safety profiles of nintedanib in patients with idiopathic pulmonary fibrosis and SSc-ILD observed in randomized controlled trails.
Expert opinion: Currently, we have two treatment options for SSc-ILD, i.e., immunosuppressants and antifibrotic agents. However, appropriate utilization of antifibrotic agents in clinical practice remains challenging, i.e., in which cases they are to be used, timing of use, how to use them properly, and whether in combination with immunosuppressants.
Keywords: Antifibrotic therapy; fibrosis; interstitial lung disease; lung function; nintedanib; scleroderma; systemic sclerosis; tyrosine kinase inhibitor.