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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.<\/jats:p>","DOI":"10.3390\/ijms24021275","type":"journal-article","created":{"date-parts":[[2023,1,9]],"date-time":"2023-01-09T03:25:58Z","timestamp":1673234758000},"page":"1275","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Endothelin-1 as a Biomarker of Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Associated with Autoimmune Diseases"],"prefix":"10.3390","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3534-8467","authenticated-orcid":false,"given":"Ver\u00f3nica","family":"Pulito-Cueto","sequence":"first","affiliation":[{"name":"Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, Avenida Cardenal Herrera Oria s\/n, Lab. 201\/202, 39011 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3612-1448","authenticated-orcid":false,"given":"Fernanda","family":"Genre","sequence":"additional","affiliation":[{"name":"Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, Avenida Cardenal Herrera Oria s\/n, Lab. 201\/202, 39011 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0548-2689","authenticated-orcid":false,"given":"Raquel","family":"L\u00f3pez-Mej\u00edas","sequence":"additional","affiliation":[{"name":"Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, Avenida Cardenal Herrera Oria s\/n, Lab. 201\/202, 39011 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8161-0462","authenticated-orcid":false,"given":"V\u00edctor Manuel","family":"Mora-Cuesta","sequence":"additional","affiliation":[{"name":"Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, Avenida Cardenal Herrera Oria s\/n, Lab. 201\/202, 39011 Santander, Spain"},{"name":"Pneumology Department, Hospital Universitario Marqu\u00e9s de Valdecilla, 39008 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5241-266X","authenticated-orcid":false,"given":"David","family":"Iturbe-Fern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, Avenida Cardenal Herrera Oria s\/n, Lab. 201\/202, 39011 Santander, Spain"},{"name":"Pneumology Department, Hospital Universitario Marqu\u00e9s de Valdecilla, 39008 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginia","family":"Portilla","sequence":"additional","affiliation":[{"name":"Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, Avenida Cardenal Herrera Oria s\/n, Lab. 201\/202, 39011 Santander, Spain"},{"name":"Rheumatology Department, Hospital Universitario Marqu\u00e9s de Valdecilla, 39008 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda","family":"Sebasti\u00e1n Mora-Gil","sequence":"additional","affiliation":[{"name":"Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, Avenida Cardenal Herrera Oria s\/n, Lab. 201\/202, 39011 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2245-4639","authenticated-orcid":false,"given":"Javier Gonzalo","family":"Ocejo-Vinyals","sequence":"additional","affiliation":[{"name":"Department of Immunology, Hospital Universitario Marqu\u00e9s de Valdecilla, 39008 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7154-1328","authenticated-orcid":false,"given":"Oreste","family":"Gualillo","sequence":"additional","affiliation":[{"name":"SERGAS (Servizo Galego de Saude) and IDIS (Instituto de Investigaci\u00f3n Sanitaria de Santiago), NEIRID Lab. 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