Metabolomics in Idiopathic Pulmonary Fibrosis: Emerging Lessons for Chronic Lung Diseases and Opportunities for Clinical Translation

Abstract

Idiopathic Pulmonary Fibrosis (IPF) is a progressive and fatal interstitial lung disease (ILD) characterized by abnormal epithelial cell behavior and excessive extracellular matrix deposition. Despite advances in understanding its molecular pathogenesis, the lack of early diagnostic biomarkers and effective targeted therapies remains a critical barrier. Metabolomics is the comprehensive profiling of low-molecular-weight metabolites and offers an emerging lens to unpack the complex metabolic reprogramming in IPF. This expert review discusses (1) current metabolomics approaches used in IPF research and (2) the key dysregulated metabolic pathways and their potential in improving diagnosis, prognostication, and treatment response monitoring. Furthermore, the review outlines the key metabolic signatures identified in non-IPF ILDs as well and compares their roles with those observed in IPF, thereby providing a broader perspective on shared and disease-specific metabolic alterations across the ILD spectrum.