Language

English

Publication Date

3-1-2025

Journal

Chinese Medical Journal Pulmonary and Critical Care Medicine

DOI

10.1016/j.pccm.2025.02.001

PMID

40226606

PMCID

PMC11993042

PubMedCentral® Posted Date

3-7-2025

PubMedCentral® Full Text Version

Post-print

Abstract

Significant advances have been made in diagnosing and treating idiopathic pulmonary fibrosis (IPF) in the last decade. The incidence and prevalence of IPF are increasing, and morbidity and mortality remain high despite the two Food and Drug Administration (FDA)-approved medications, pirfenidone and nintedanib. Hence, there is an urgent need to develop new diagnostic tools and effective therapeutics to improve early, accurate diagnosis of IPF and halt or reverse the progression of fibrosis with a better safety profile. New diagnostic tools such as transbronchial cryobiopsy and genomic classifier require less tissue and generally have good safety profiles, and they have been increasingly utilized in clinical practice. Advances in artificial intelligence-aided diagnostic software are promising, but challenges remain. Both pirfenidone and nintedanib focus on growth factor-activated pathways to inhibit fibroblast activation. Novel therapies targeting different pathways and cell types (immune and epithelial cells) are being investigated. Biomarker-based personalized medicine approaches are also in clinical trials. This review aims to summarize recent diagnostic and therapeutic development in IPF.

Keywords

Idiopathic pulmonary fibrosis, Biomarkers, Cryobiopsy, Genomic classifier, Clinical trials

Published Open-Access

yes

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