Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis.

ABSTRACT: Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among patients with IPF over time and are the only two disease-modulating pharmacological agents approved by regulatory agencies and available for clinical use worldwide. With the evolved standard of care for interstitial lung disease evaluation including routine use of high-resolution computed tomography, fibrotic lung diseases other than IPF are increasingly recognised. In addition, it is becoming evident that genetic and pathophysiological mechanisms as well as disease behaviour in patients manifes

SUBMITTER: Collins BF 

PROVIDER: S-EPMC9489066 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature