Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigación Marqués de Valdecilla, Avenida Cardenal Herrera Oria s/n, Lab. 201/202, 39011 Santander, Spain.
Pneumology Department, Hospital Universitario Marqués de Valdecilla, 39008 Santander, Spain.
Int J Mol Sci. 2023 Jan 9;24(2):1275. doi: 10.3390/ijms24021275.
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.
本研究旨在确定内皮素-1（ET-1）在多种血管和纤维异常中的作用，作为间质性肺病（ILD）的生物标志物，以及在特发性肺纤维化（IPF）和自身免疫性疾病相关ILD（AD-ILD）的鉴别诊断中的应用，使用ILD 患者的大型和明确队列。共纳入 112 例 IPF 患者、91 例 AD-ILD 患者（28 例类风湿关节炎（RA）、26 例系统性硬化症、20 例特发性炎性肌病和 17 例自身免疫性特征性间质性肺炎）和 44 例健康对照者。采用酶联免疫吸附试验测定 ET-1 血清水平。与对照组相比，IPF 患者的 ET-1 水平明显升高。同样，当按 AD 类型对整个队列进行分层时，AD-ILD 患者的 ET-1 水平也高于对照组。无论潜在的 AD 如何，在 IPF 和 AD-ILD 患者中均发现相似的 ET-1 水平。有趣的是，升高的 ET-1 水平与 IPF 和 RA-ILD 患者的肺功能恶化相关。我们的研究支持血清 ET-1 可作为 ILD 的生物标志物，尽管它无助于 IPF 和 AD-ILD 之间的鉴别诊断。此外，ET-1 水平可能与 ILD 严重程度相关。
