Pulmonary fibrosis encompasses a spectrum of interstitial lung diseases (ILDs) characterized by chronic inflammation, aberrant tissue remodeling, and excessive extracellular matrix deposition, ultimately leading to respiratory failure. Increasing evidence highlights the pivotal role of the immune system—not merely as a bystander, but as an active driver—of fibrogenesis. Both innate and adaptive immune cells contribute to the initiation, perpetuation, and resolution of fibrosis. Macrophage plasticity, lymphocyte activation, neutrophil-derived mediators, and dendritic cell signaling have all been implicated in shaping the fibrotic niche. Despite anti-fibrotic therapies improving outcomes in some patients, our understanding of the immunological underpinnings of pulmonary fibrosis remains incomplete. A deeper exploration of immune cell interactions in the fibrotic lung may unlock novel diagnostic biomarkers and therapeutic targets.

This Research Topic aims to unravel the immunopathological mechanisms that underpin pulmonary fibrosis, with a special focus on the roles of immune cells in disease onset, progression, and resolution. Although anti-fibrotic agents such as nintedanib and pirfenidone have improved survival in idiopathic pulmonary fibrosis (IPF), they do not address the heterogeneity of immune-driven fibrotic pathways. Recent advances in spatial transcriptomics, single-cell RNA sequencing, and immunophenotyping have revealed distinct immune cell populations and profibrotic cytokine networks active within fibrotic lesions. Furthermore, conditions such as connective tissue disease-associated ILD (CTD-ILD), fibrotic hypersensitivity pneumonitis, and microaspiration-related fibrosis display diverse immune signatures, suggesting that targeted modulation of immune responses may offer personalized therapeutic opportunities. By collecting original research, reviews, and translational studies, this Research Topic will provide an integrated view of the immune–fibrotic interface and foster dialogue across immunology, pulmonology, and pathology disciplines.

We welcome contributions that explore the immunobiology of pulmonary fibrosis across experimental models, clinical samples, and computational approaches. The Research Topic invites manuscripts that address, but are not limited to, the following themes:

• Role of macrophage polarization and fibroblast crosstalk in lung fibrosis
• Adaptive immunity and T/B cell subsets in fibrotic ILDs
• Neutrophil extracellular traps and their impact on matrix remodeling
• Immune checkpoints and regulatory pathways in fibrogenesis
• Biomarker discovery and immune profiling in IPF and secondary fibroses
• Effects of environmental triggers (e.g., infection, microaspiration) on immune activation
• Immunotherapeutic approaches for fibrotic lung disease

We encourage submission of original research articles, systematic or narrative reviews, perspectives, and hypothesis-driven opinion pieces.

Are you interested in contributing a manuscript to this Research Topic?

Immune Cell Dynamics in Pulmonary Fibrosis: From Mechanisms to Therapeutic Targets