Cellular and Molecular Drivers of Pulmonary Fibrosis: From Immune Dysregulation to Translational Biomarkers and Clinical Phenotypes

The landscape of fibrotic interstitial lung diseases (ILDs) is undergoing a significant paradigm shift. While traditionally viewed through the lens of terminal scarring, modern research now recognizes fibrosis as a highly dynamic process. It is driven by a complex, multifaceted interplay between chronic epithelial injury, persistent fibroblast activation, and profound immune dysregulation. Conditions such as Idiopathic Pulmonary Fibrosis (IPF), sarcoidosis, and the emerging challenge of post-COVID-19 fibrosis share common pathogenic pathways while maintaining distinct clinical trajectories. To effectively tackle these progressive conditions, there is a critical need to bridge the gaps between basic molecular mechanisms, cellular communication networks, and heterogeneous clinical manifestations. Understanding these shared and unique pathways is essential for shifting the treatment paradigm from broad-spectrum management to targeted, individualized patient care.

The goal of this Research Topic is to provide a multidisciplinary platform that seamlessly connects the bench to the bedside. By integrating basic molecular insights with clinical reality, this collection aims to shed light on the specific mechanisms that dictate disease progression and accelerate the discovery of precision biomarkers. We seek to explore three key pillars: molecular and cellular mechanics (including epithelial-to-mesenchymal transition and upstream alarmins), immune profiling via advanced methods (such as extracellular vesicle analysis), and clinical translation through high-resolution imaging and phenotypic mapping. Ultimately, this collection aims to foster the development of targeted therapies and improve the management of patients suffering from progressive fibrotic lung diseases.

This Research Topic welcomes contributions that improve our understanding of the cellular and molecular drivers of pulmonary fibrosis. Potential areas of interest include, but are not limited to:

• In-vitro and in-vivo models of lung epithelial injury and repair.
• The role of alarmins and innate lymphoid cells in fibrotic signaling.
• Extracellular vesicles as mediators of lung remodeling and potential liquid biopsies.
• Immune cell dynamics in the bronchoalveolar lavage (BAL) and peripheral blood of idiopathic- and secondary-ILD patients.
• Integration of molecular signatures with clinical functional decline and imaging.
• Mechanistic insights into sarcoidosis, IPF, and post-infectious pulmonary fibrosis.

We welcome the submission of different article types to this collection, especially Original Research, Reviews, Mini-Reviews, and Perspective articles. We highly encourage studies combining molecular laboratory expertise with clinical and translational observations. Even though abstract submission is not mandatory, we encourage all interested researchers to submit a manuscript summary (abstract) before submitting their full manuscript. These short summaries do not have to coincide with the final abstract of the manuscript but will serve as a tool for the editorial team to provide early feedback and ensure alignment with the scope of this collection.

Article types and fees

This Research Topic accepts the following article types, unless otherwise specified in the Research Topic description:

• Brief Research Report
• Case Report
• Data Report
• Editorial
• FAIR² Data
• Hypothesis and Theory
• Methods
• Mini Review
• Opinion
• ... View all formats

Articles that are accepted for publication by our external editors following rigorous peer review incur a publishing fee charged to Authors, institutions, or funders.

Article types

This Research Topic accepts the following article types, unless otherwise specified in the Research Topic description:

• Brief Research Report
• Case Report
• Data Report
• Editorial
• FAIR² Data
• Hypothesis and Theory
• Methods
• Mini Review
• Opinion
• Original Research
• Perspective
• Review
• Systematic Review
• Technology and Code

Keywords: Pulmonary fibrosis, Interstitial lung disease, Epithelial-mesenchymal transition, Extracellular vesicles, Immune dysregulation, Alarmins, Post-COVID fibrosis

Important note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.