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Updated: Jul 17, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Assessment of plasma endothelin level measurement in systemic sclerosis]
Insights
Endothelin-1 (ET-1) levels are elevated in systemic sclerosis (SSc) patients with pulmonary arterial hypertension (PAH). Higher ET-1 correlates with pulmonary arterial pressure, aiding PAH detection in SSc.
Area of Science:
- Cardiovascular Research
- Rheumatology
- Pulmonology
Background:
- Endothelin (ET)-1 is implicated in the pathogenesis of systemic sclerosis (SSc).
- Understanding ET-1's role in SSc complications like pulmonary arterial hypertension (PAH) is crucial.
Purpose of the Study:
- To assess plasma endothelin (ET)-1 levels in systemic sclerosis (SSc) patients.
- To correlate ET-1 levels with clinical presentation and complications, specifically pulmonary arterial hypertension (PAH).
Main Methods:
- Plasma ET-1 levels were measured in 63 SSc patients and 17 healthy controls.
- Pulmonary function tests, HRCT, echocardiography, and right heart catheterization were used to screen for and confirm PAH.
- Statistical analyses, including ROC curve, were performed to evaluate ET-1's diagnostic utility.
Main Results:
- ET-1 levels were not significantly higher in SSc patients compared to controls.
- Significantly higher ET-1 levels were observed in SSc patients with PAH (p=0.04) and those with anticentromere antibodies (p=0.03).
- A positive linear correlation was found between ET-1 levels and systolic pulmonary arterial pressure (r=0.34, p=0.013).
Conclusions:
- Plasma ET-1 levels are elevated in SSc patients with PAH and anticentromere antibodies.
- ET-1 levels correlate with pulmonary arterial pressure, suggesting its potential as a biomarker.
- Further prospective studies are warranted to evaluate ET-1 for detecting and monitoring pulmonary hypertension in SSc.
Purpose:
According to current knowledge, endothelin (ET)-1 plays an important role in the pathogenesis of systemic sclerosis (SSc). We assessed ET plasma levels in SSc patients according to the clinical presentation and the presence of complications such as pulmonary arterial hypertension (PAH).
Methods:
Sixty-three consecutive patients with SSc were included. The control group included 17 healthy patients. ET plasma level was determined for all patients. Pulmonary function test and pulmonary high resolution computed tomography were performed in 44 patients and echocardiography in 51 patients, to screen for PAH, always confirmed by a right heart catheterization.
Results:
ET plasmatic levels were higher in SSc patients than in healthy group subjects but the difference was not significant (3.72+/-1.13 vs 3.40+/-0.71 pmol/l, p=0.27). ET plasmatic levels were significantly higher in patients with PAH than in patients without PAH (4.28+/-0.65 vs 3.62+/-1.07 pmol/l, p=0.04) and in patients with anticentromere antibodies (3.96+/-1.11 vs 3.19+/-1.12 pmol/l, p=0.03). There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure (r=0.34, p=0.013). The best cut-off value for ET plasmatic level to discriminate patients affected by PAH was determined by ROC curve method: 4.1 pmol/l (sensibility 85.7%, specificity 66%).
Conclusion:
ET plasmatic levels were higher in SSc patients affected by PAH and patients with anticentromere antibodies. There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure. Assessment of ET plasmatic levels for detection and monitoring of pulmonary hypertension during SSc is warranted in larger prospective studies.
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