Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 24, Issue 2, Pages -Publisher
MDPI
DOI: 10.3390/ijms24021275
Keywords
interstitial lung disease; idiopathic pulmonary fibrosis; interstitial lung disease associated with autoimmune diseases; biomarker; endothelin-1
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The aim of this study was to investigate the role of endothelin-1 (ET-1) as a biomarker of interstitial lung disease (ILD), and to differentiate between idiopathic pulmonary fibrosis (IPF) and ILD associated with autoimmune diseases (AD-ILD). The study included a large cohort of ILD patients and healthy controls, and found that ET-1 levels were increased in IPF and AD-ILD patients compared to controls. However, ET-1 levels could not differentiate between IPF and AD-ILD, and were associated with worse lung function in IPF and RA-ILD patients.
The aim of this study was to determine the role of endothelin-1 (ET-1), a molecule involved in multiple vascular and fibrosing abnormalities, as a biomarker of interstitial lung disease (ILD), as well as its use for the differential diagnosis between idiopathic pulmonary fibrosis (IPF) and ILD associated with autoimmune diseases (AD-ILD), using a large and well-defined cohort of patients with ILD. A total of 112 patients with IPF, 91 patients with AD-ILD (28 rheumatoid arthritis (RA), 26 systemic sclerosis, 20 idiopathic inflammatory myositis and 17 interstitial pneumonia with autoimmune features) and 44 healthy controls were included. ET-1 serum levels were determined by enzyme-linked immunosorbent assay. A significant increase in ET-1 levels was found in patients with IPF compared to controls. Likewise, AD-ILD patients also showed higher ET-1 levels than controls when the whole cohort was stratified by the type of AD. Similar ET-1 levels were found in IPF and AD-ILD patients, regardless of the underlying AD. Interestingly, increased ET-1 levels were correlated with worse lung function in IPF and RA-ILD patients. Our study supports that serum ET-1 may be useful as a biomarker of ILD, although it could not help in the differential diagnosis between IPF and AD-ILD. Moreover, ET-1 levels may be associated with ILD severity.
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