Biomarkers in idiopathic pulmonary fibrosis
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic, debilitating, fibrotic lung disease leading to respiratory failure and ultimately to death. Being the prototype of interstitial lung diseases, IPF is characterized by marked heterogeneity regarding its clinical course. Despite significant progress in the understanding of its pathogenesis, we still cannot reliably predict the course of the disease and the response to treatment of an individual patient. Non-invasive biomarkers, in particular serum biomarkers, for the (early) diagnosis, differential diagnosis, prognosis and prediction of therapeutic response are urgently needed. Numerous molecules involved in alveolar epithelial cell injury, fibroproliferation and matrix remodeling as well as immune regulation have been proposed as potential biomarkers. Furthermore, genetic variants of TOLLIP, MUC5B, and other genes are associated with a differential response to treatment and with the development and/or the prognosis of IPF. Additionally, the bacterial signature in IPF lungs, as shown from microbiome analyses, as well as mitochondrial DNA seem to have promising roles as biomarkers. Moreover, combination of multiple biomarkers may identify comprehensive biomarker signatures in IPF patients. However, there is still a long way until these potential biomarkers complete or substitute for the clinical and functional parameters currently available for IPF.
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In the context of IPF, biomarkers can be classified based on their association with alveolar epithelial dysfunction, ECM remodelling and fibroproliferation, and immune dysfunction.
“In the context of IPF, biomarkers can be classified based on their association with alveolar epithelial dysfunction (such as Krebs von den Lungen-6, surfactant proteins and mucin-5B), ECM remodelling and fibroproliferation (including MMPs, lysyl oxidase-like proteins, periostin and OPN) and immune dysfunction (such as CC motif chemokine ligand-18 and YKL-40) (Drakopanagiotakis et al., 2018).”
From PDE4B inhibition by nerandomilast: Effects on lung fibrosis and transcriptome in fibrotic rats and on biomarkers in human lung epithelial cells (2024) · cites this paper as Drakopanagiotakis et al., 2018
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“Several molecules implicated in alveolar epithelial cell injury, fibroproliferation, matrix remodelling and immune regulation have been suggested as potential biomarkers (Drakopanagiotakis et al., 2018).”
Guo, H., Sun, J., Zhang, S., Nie, Y., Zhou, S., & Zeng, Y. (2023) · Frontiers in Pharmacology · doi:10.3389/fphar.2023.1205948 · Cited by 41 · see citing works
- Authors
- Drakopanagiotakis, F., Wujak, L., Wygrecka, M., & Markart, P.
- Affiliations
- Klinikum Fulda (DE)
- Universities of Giessen and Marburg Lung Center (DE)
- Justus-Liebig-Universität Gießen (DE)
- Corresponding author
- Markart, P.
- Venue
- Matrix Biology
- Publisher
- Elsevier BV
- Published
- 2018-08
- Volume
- 68-69
- Pages
- 404-421
- ISSN
- 0945-053X (ISSN-L)
- OpenAlex
- W2790507111
- Cited by
- 132
- References
- 19
- Citations by year
- 2026: 5 · 2025: 11 · 2024: 26 · 2023: 18 · 2022: 21 · 2021: 18 · 2020: 17 · 2019: 9 · 2018: 7
- FWCI
- 2.28
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Matrix Biology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- idiopathic pulmonary fibrosis, biomarkers, serum biomarkers, interstitial lung disease, disease prognosis, treatment response prediction, Tollip, MUC5B, genetic variants, lung microbiome, mitochondrial DNA, alveolar epithelial cell injury, extracellular matrix remodeling, biomarker signatures
- MeSH terms
- Gastrointestinal Microbiome, Diagnosis, Differential, DNA, Mitochondrial, Humans, Prognosis, Biomarkers, Disease Progression, Early Diagnosis, Intracellular Signaling Peptides and Proteins, Idiopathic Pulmonary Fibrosis, Mucin-5B
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