An IPF-like disease course in disorders other than IPF: how can this be anticipated, recognized, and managed?

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF)-like chronic disease progression despite treatment cannot be predicted with confidence in interstitial lung diseases (ILDs) other than IPF at the time of diagnosis.

Areas covered: We review key determinants of a progressive fibrotic phenotype, at initial diagnosis of an ILD other than IPF. Medline literature searches (2000 to 2020) were undertaken with regard to the issues discussed in this review.

Expert opinion: The definition of the progressive fibrotic phenotype in non-IPF patients should remain real world, with a conclusion reached by an experienced clinician that progression has occurred despite the use of appropriate historical therapies, on a case by case basis. There is an urgent need for pathogenetic studies to identify pathways and genetic predilections that are common to chronic progressive fibrosis across different diseases. Efforts should also be focused on the identification of the progressive fibrotic phenotype at first presentation, potentially through a combination of CT and biopsy evaluation and the definition of a biomarker profile associated with subsequent disease progression. Recent anti-fibrotic trials of non-IPF disorders should lead to trials of combination regimens of anti-fibrotic agents and immunomodulatory or other therapies specific to individual diseases.

Keywords: Diagnosis; disease behavior classification; disease severity; progressive fibrotic phenotype; response to treatment; treatment.