Antifibrotic therapy in progressive pulmonary fibrosis: a review of recent advances

ABSTRACT

Introduction

Progressive pulmonary fibrosis (PPF) is a manifestation of a heterogenous group of underlying interstitial lung disease (ILD) diagnoses, defined as non-idiopathic pulmonary fibrosis (IPF) progressive fibrotic ILD meeting at least two of the following criteria in the previous 12 months: worsening respiratory symptoms, absolute decline in forced vital capacity (FVC) more than or equal to 5% and/or absolute decline in diffusing capacity for carbon monoxide (DLCO) more than or equal to 10% and/or radiological progression.

Areas covered

The authors subjectively reviewed a synthesis of literature from PubMed to identify recent advances in the diagnosis and characterisation of PPF, treatment recommendations, and management challenges. This review provides a comprehensive summary of recent advances and highlights future directions for the diagnosis, management, and treatment of PPF.

Expert Opinion

Recent advances in defining the criteria for PPF diagnosis and licensing of treatment are likely to support further characterisation of the PPF patient population and improve our understanding of prevalence. The diagnosis of PPF remains challenging with the need for a specialised ILD multidisciplinary team (MDT) approach. The evidence base supports the use of immunomodulatory therapy to treat inflammatory ILDs and antifibrotic therapy where PPF develops. Treatment needs to be tailored to the specific underlying disease and determined on a case-by-case basis.

KEYWORDS:

• Antifibrotics
• nintedanib
• pirfenidone
• pulmonary fibrosis
• progressive pulmonary fibrosis
• interstitial lung disease

Article highlights

• Recent advances in defining the criteria for progressive pulmonary fibrosis (PPF) and licensing of treatment are likely to support further characterisation of the PPF patient population and improve our understanding of prevalence.

• The accurate and timely diagnosis of PPF remains challenging with the need for a specialised interstitial lung disease (ILD) multidisciplinary (MDT) approach.

• Robust clinical trial data to evaluate the efficacy and safety of new and current therapies is lacking, in part due to the relatively low prevalence of specific fibrosing ILD subtypes.

• The evidence base supports use of immunomodulatory therapy to treat inflammatory ILDs and antifibrotic therapy where PPF develops. Treatment needs to be tailored to the specific underlying disease and determined on a case-by-case basis.

• We expect earlier identification of progression and improved diagnosis of PPF with technological advances and use of biomarkers to guide clinical practice.

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.

Reviewer disclosures

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

Additional information

Funding

This paper was not funded.