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

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.

KEYWORDS:

• Interstitial lung disease
• progressive fibrosing
• antifibrotic
• immunosuppressive
• multidisciplinary team
• phenotype

Article highlights

• There is a subset of patients with interstitial lung disease (ILD) who demonstrate a progressive phenotype, similar to patients with idiopathic pulmonary fibrosis (IPF), manifested by worsening symptoms, lung function and progression of fibrotic changes on imaging despite traditional immunomodulatory treatments leading to an increased risk of mortality.

• Mechanistic and clinical parallels between IPF and non-IPF progressive fibrosing ILDs (PF-ILD) suggest that antifibrotic therapy may be a viable therapeutic option in this group and there have been recent and ongoing clinical trials assessing the safety and efficacy of antifibrotic therapy for patients with non IPF PF-ILD.

• Antifibrotics for patients progressing on traditional treatments would be a welcome strategy for non IPF PF-ILD patients but it will also introduce challenges.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

This paper was not funded.