• Philipp Markart, Fotios Drakopanagiotakis, and Malgorzata Wygrecka.
• Medizinische Klinik V, Campus Fulda, Universitätsmedizin Marburg.
• Dtsch. Med. Wochenschr. 2021 Feb 1; 146 (3): 181-184.

AbstractInterstitial 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.Thieme. All rights reserved.

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