The extended utility of antifibrotic therapy in progressive fibrosing interstitial lung disease

Abstract

ABSTRACT Introduction The approval of two antifibrotic treatment agents for delaying disease progression in idiopathic pulmonary fibrosis (IPF), has prompted researchers to look at expanding the role of antifibrotic therapy to other fibrosing interstitial lung disease (ILD). Similarities in the pathological mechanisms that lead to the development of IPF have been implicated in other progressive fibrosing ILD (PF-ILD) such as chronic hypersensitivity pneumonitis, connective tissues disease associated ILD, sarcoidosis, occupational ILD and idiopathic non-specific interstitial pneumonia (iNSIP). This has prompted the rationale to use antifibrotic therapy to target similar molecular pathways in these diseases. Areas covered This review will summarise the available evidence from randomised controlled trials that have evaluated the use of antifibrotic therapy in PF-ILD outside the realm of IPF. Expert opinion There is promising data for antifibrotic therapy as a therapeutic option for non IPF PF-ILD. The new therapy option does provide some challenges that need to be addressed such as timing of initiation of therapy, clarifying the strategy for overlap or combination with existing immunosuppressive therapies and potential drug interactions. There is an unmet need to determine accurate predictors of disease progression to allow early intervention for the preservation of lung function and mortality reduction.