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Publications

Journal Article Review Epublish

PubMed: 42660607 DOI: 10.1183/13993003.00258-2026

The role of inflammation in the pathogenesis of pulmonary fibrosis: mechanisms and treatment implications.

Eur Respir J 68(4)

Authors: Nazanin Nazemi, Bruno Crestani, Doumet Georges Helou, Oliver Distler, Janine Gote-Schniering, Anna-Maria Hoffmann Vold, Carlos Machahua, Martin Kolb, Manuela Funke-Chambour

Affiliations

  • Department for Pulmonary Medicine, Allergology and Clinical Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. Lung Precision Medicine, Department for BioMedical Research, University of Bern, Bern, Switzerland.
  • Université Paris Cité, AP-HP, Hôpital Bichat-Claude Bernard, Respiratory Medicine, Paris, France.
  • Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
  • Department for Pulmonary Medicine, Allergology and Clinical Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. Lung Precision Medicine, Department for BioMedical Research, University of Bern, Bern, Switzerland. Department of Rheumatology and Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
  • Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. Department of Rheumatology, Oslo University Hospital, Oslo, Norway.
  • Department of Medicine, Firestone Institute for Respiratory Health, McMaster University, Hamilton, ON, Canada.
  • Department for Pulmonary Medicine, Allergology and Clinical Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. Lung Precision Medicine, Department for BioMedical Research, University of Bern, Bern, Switzerland. Division of Pulmonology, Department of Medicine, Lausanne University Hospital (CHUV) and University of Lausanne, Lausanne, Switzerland.

Abstract

Fibrotic interstitial lung diseases (fILDs) encompass a heterogeneous group of interstitial lung diseases and may arise from persistent inflammation caused by systemic disease or environmental exposures. Idiopathic pulmonary fibrosis, the prototypical fILD, is characterised by progressive fibrosis, limited inflammation, and a high mortality rate. In contrast, other fILDs display variable contributions of fibrosis and inflammation that are only partially determined by the underlying diagnosis. Within most fILDs, a broad spectrum of disease phenotypes can be observed.Inflammation and fibrosis are not discrete, sequential events, but rather dynamically intertwined processes. The nature and relative importance of these interactions vary according to the underlying aetiology. Inflammation can function as both a driver and a consequence of fibrotic remodelling, establishing self-perpetuating feed-forward loops, yet also playing a role in fibrosis resolution and control. As therapeutic strategies for predominantly fibrotic versus inflammatory interstitial lung diseases differ substantially, improved understanding of the pathomechanisms that drive fibrosis is essential for optimising personalised treatment approaches.Fibrotic and inflammatory signalling pathways show considerable overlap, and their interaction is central to disease initiation and progression. Elucidating the mechanisms that govern cellular responses to diverse injurious stimuli provides an opportunity to refine patient stratification and tailor therapies to improve outcomes. Insights from experimental models of pulmonary fibrosis and translational research have significantly advanced our understanding of these processes and are reviewed in this article, together with current therapeutic advances and future directions.

MeSH

Lung, Fibrosis, Respiration, Pulmonary Fibrosis, Therapeutics, Lung Diseases, Lung Diseases, Interstitial