Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Gabrielle Y. Liu, G. R.Scott Budinger, Jane E. Dematte*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

34 Scopus citations

Abstract

Similarly to idiopathic pulmonary fibrosis (IPF), other interstitial lung diseases can develop progressive pulmonary fibrosis (PPF) characterized by declining lung function, a poor response to immunomodulatory therapies, and early mortality. The pathophysiology of disordered lung repair involves common downstream pathways that lead to pulmonary fibrosis in both IPF and PPF. The antifibrotic drugs, such as nintedanib, are indicated for the treatment of IPF and PPF, and new therapies are being evaluated in clinical trials. Clinical, radiographic, and molecular biomarkers are needed to identify patients with PPF and subgroups of patients likely to respond to specific therapies. This article reviews the evidence supporting the use of specific therapies in patients with IPF and PPF, discusses agents being considered in clinical trials, and considers potential biomarkers based on disease pathogenesis that might be used to provide a personalized approach to care.

Original languageEnglish (US)
Article numbere066354
JournalThe BMJ
DOIs
StateAccepted/In press - 2022

Funding

Funding: GYL is supported by NIH grant F32-HL162318 and North Western University’s Lung Sciences Training Program 5T32HL076139-17. GRSB is supported by supported by NIH grants ES013995, HL071643, and AG049665 and the Veterans Administration grant BX000201.

ASJC Scopus subject areas

  • General Medicine

Fingerprint

Dive into the research topics of 'Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis'. Together they form a unique fingerprint.

Cite this