Treatment with nintedanib, a tyrosine kinase inhibitor, slowed the progression of interstitial lung disease (ILD) associated with systemic sclerosis (SSc) in a 52-week randomized placebo-controlled trial. The adjusted annual rate of decline in forced vital capacity was lower in the nintedanib-treated group than in the placebo-treated group (P = 0.04), although no clinical benefits for other manifestations of SSc were observed. The adverse-event profile was similar to that reported in previous trials of patients with idiopathic pulmonary fibrosis; the rate of gastrointestinal adverse events, including diarrhoea, was higher in the nintedanib group than in the placebo group.