Nintedanib (Ofev™), an oral triple kinase inhibitor targeting pro-fibrotic 
pathways, has been used for treatment of idiopathic pulmonary fibrosis (IPF). 
Based on positive results from phase III, placebo-controlled, randomized 
comparative clinical trial conducted in patients with systemic 
sclerosis-associated interstitial lung disease (SSc-ILD), nintedanib received 
marketing approval in the United States and Japan for the treatment of SSc-ILD. 
Nintedanib significantly reduced the annual rate of decline in forced vital 
capacity over 52 weeks compared with placebo. The safety profiles observed in 
this trial were consistent with those reported in IPF patients, and the most 
common adverse events were gastrointestinal disorders, including diarrhea, 
nausea, and vomiting, which sometimes lead to discontinuation or permanent dose 
reduction of nintedanib. In contrast, serious adverse events were infrequent and 
were related mostly to worsening of cardiopulmonary involvement of SSc. This 
review summarizes the milestones in development of nintedanib leading to the 
approval for the treatment of SSc-ILD, and covers mechanisms of action, efficacy 
results and safety profiles, and future perspectives of nintedanib.