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Updated: Nov 30, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Elevated Circulatory Levels of Microparticles Are Associated to Lung Fibrosis and Vasculopathy During Systemic
Damien Leleu1,2, Emeline Levionnois1, Paoline Laurent1
1University of Bordeaux, CNRS, ImmunoConcEpT, UMR 5164, Bordeaux, France.
Background:
Microparticles (MPs) are vesicular structures that derive from multiple cellular sources. MPs play important roles in intercellular communication, regulation of cell signaling or initiation of enzymatic processes. While MPs were characterized in Systemic Sclerosis (SSc) patients, their contribution to SSc pathogenesis remains unknown. Our aim was to investigate the potential role of MPs in SSc pathophysiology and their impact on tissue fibrosis.
Methods:
Ninety-six SSc patients and 37 sex-matched healthy donors (HD) were enrolled in this study in order to quantify and phenotype their plasmatic MPs by flow cytometry. The ability of MPs purified from SSc patients and HD controls to modulate fibroblast's extra-cellular matrix genes expression was evaluated in vitro by reverse transcriptase quantitative polymerase chain reaction.
Results:
SSc patients exhibited a higher concentration of circulatory MPs compared to HD. This difference was exacerbated when we only considered patients that were not treated with methotrexate or targeted disease-modifying antirheumatic drugs. Total circulatory MPs were associated to interstitial lung disease, lung fibrosis and diminished lung functional capacity, but also to vascular involvement such as active digital ulcers. Finally, contrary to HD MPs, MPs from SSc patients stimulated the production of extracellular matrix by fibroblast, demonstrating their profibrotic potential.
Conclusions:
In this study, we provide evidence for a direct profibrotic role of MPs from SSc patients, underpinned by strong clinical associations in a large cohort of patients.
Insights
Microparticles (MPs) from Systemic Sclerosis (SSc) patients are more abundant and promote fibrosis. These findings link MPs to SSc severity and tissue damage, suggesting a direct role in disease progression.
Area of Science:
- Immunology
- Cell Biology
- Rheumatology
Background:
- Microparticles (MPs) are cell-derived vesicles involved in intercellular communication.
- While MPs are found in Systemic Sclerosis (SSc) patients, their specific role in SSc pathogenesis is unclear.
- This study investigates the contribution of MPs to SSc pathophysiology and tissue fibrosis.
Purpose of the Study:
- To quantify and phenotype plasmatic MPs in SSc patients and healthy donors (HD).
- To evaluate the in vitro effect of MPs on fibroblast extracellular matrix gene expression.
- To determine the association of MPs with SSc clinical manifestations and disease severity.
Main Methods:
- Flow cytometry was used to analyze MPs from 96 SSc patients and 37 HD.
- In vitro assays assessed the impact of purified MPs on fibroblast extracellular matrix gene expression.
- Correlation analyses examined the association between MP levels and clinical parameters in SSc.
Main Results:
- SSc patients had higher circulatory MP concentrations than HD, especially those untreated with certain drugs.
- Elevated MP levels correlated with interstitial lung disease, lung fibrosis, reduced lung function, and vascular issues like digital ulcers.
- MPs from SSc patients, unlike those from HD, stimulated fibroblast extracellular matrix production, indicating profibrotic potential.
Conclusions:
- This study provides evidence for a direct profibrotic role of MPs in SSc.
- The findings highlight a strong association between MPs and clinical manifestations in a large SSc cohort.
- MPs represent a potential therapeutic target for mitigating fibrosis in Systemic Sclerosis.
