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The Intricate Relationship Between Pulmonary Fibrosis and Thrombotic Pathology: A Narrative Review
Giovanni Cenerini1, Davide Chimera1, Marta Pagnini2
1UO Pneumologia, Azienda Ospedaliero-Universitaria Pisana, 56124 Pisa, Italy.
Idiopathic pulmonary fibrosis (IPF) increases thrombosis risk. Extracellular vesicles (EVs) link fibrosis and coagulation, offering new therapeutic targets for managing this complex relationship.
Area of Science:
- Pulmonary Medicine
- Hematology
- Cell Biology
Background:
- Idiopathic pulmonary fibrosis (IPF) is linked to higher rates of thrombotic events and mortality.
- A complex, bidirectional relationship exists between pulmonary fibrosis and thrombosis.
- Extracellular vesicles (EVs) are increasingly recognized as key mediators in interstitial lung diseases (ILDs).
Purpose of the Study:
- To review the epidemiological evidence, pathogenetic mechanisms, and therapeutic strategies concerning the interplay between pulmonary fibrosis and thrombosis.
- To highlight the role of extracellular vesicles (EVs) in linking fibrosis and coagulation.
- To discuss emerging therapeutic implications and future research directions.
Main Methods:
- Literature review of epidemiological studies, pathogenetic mechanisms, and clinical trials.
- Focus on the role of coagulation factors, thrombotic pathways, and extracellular vesicles (EVs).
- Analysis of preclinical and early clinical data for novel therapeutic agents and EV-based approaches.
Main Results:
- Coagulation factors promote fibrosis, while fibrosis activates thrombotic pathways.
- Retrospective studies suggest anticoagulant benefits in IPF, though prospective trials face challenges.
- EVs act as crucial mediators in ILDs, contributing to both fibrosis and coagulation.
Conclusions:
- EVs are central to the pathogenesis of ILDs, linking fibrosis and thrombosis.
- Therapeutic strategies targeting EVs, novel anticoagulants, and profibrinolytic agents show promise.
- Further research is needed to optimize treatments, identify biomarkers, and understand EV roles in ILDs.
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