Single cell transcriptomics in a treatment-segregated cohort exposes a STAT3-regulated therapeutic gap in idiopathic pulmonary fibrosis.

ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic pulmonary disease with unknown etiology. Since approved idiopathic pulmonary fibrosis (IPF) drugs only slow disease progression, novel therapeutics are required that improve clinical outcomes. Here, we report a single cell RNA-Seq and regulatory network analysis of the largest IPF cohort assembled to date. Segregating this cohort based on status of treatment with approved antifibrotics (untreated, nintedanib- and pirfenidone-treated), we describe for the first time the transcriptional landscape of untreated IPF across 40 lung cell types, and the elements of this program that are impacted by approved antifibrotics. On average, 60% of the untreated IPF-dysregulated transcriptome is refractory to treatment with these drugs, a trans

SUBMITTER: McKenna NJ 

PROVIDER: S-EPMC12262394 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature