Interstitial lung diseases (ILD) encompass more than 200 disorders of known and unknow etiology. Until recently, the antifibrotic drugs
nintedanib and
pirfenidone had only been approved for the treatment of
idiopathic pulmonary fibrosis (IPF), but not for other ILD. In the randomized, double-blind, placebo-controlled SENSCIS trial, the efficacy and safety of
nintedanib was investigated in patients with ILD associated with
systemic sclerosis (SSc-ILD).
Nintedanib significantly reduced the annual rate of decline in FVC compared with placebo leading to the approval of
nintedanib for the treatment of SSc-ILD. Very recently,
nintedanib has got approval for chronic fibrosing ILD with a progressive phenotype based on the data from the INBUILD trial. In this randomized, double-blind, placebo-controlled, phase III trial, patients with chronic
hypersensitivity pneumonitis, idiopathic non-specific
interstitial pneumonia, unclassifiable ILD, autoimmune ILD,
sarcoidosis, and others were included, and
nintedanib slowed the annual decline of pulmonary function by 57 % in these patients. Promising data are also available for
pirfenidone in the treatment of patients with progressive, non-IPF lung
fibrosis and unclassifiable progressive fibrosing ILD. In this article, we summarize the new approvals of antifibrotic drugs in non-IPF ILD, the results from the underlying clinical trials and the clinical implications.