{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T10:43:35Z","timestamp":1787309015010,"version":"build-2736575974"},"reference-count":39,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2023,9,7]],"date-time":"2023-09-07T00:00:00Z","timestamp":1694044800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI21\/00735"],"award-info":[{"award-number":["PI21\/00735"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["FIS19\/01152"],"award-info":[{"award-number":["FIS19\/01152"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["BECA SEPAR 2018\/792"],"award-info":[{"award-number":["BECA SEPAR 2018\/792"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["BECA SEPAR Innovaci\u00f3n"],"award-info":[{"award-number":["BECA SEPAR Innovaci\u00f3n"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>(1) The role of the immune response in the pathogenesis of idiopathic pulmonary fibrosis (IPF) remains controversial. We hypothesized that peripheral blood immune phenotypes will be different in IPF patients and may relate to the disease severity and progression. (2) Whole blood flow cytometry staining was performed at diagnosis in 32 IPF patients, and in 32 age- and smoking-matched healthy controls. Thirty-one IPF patients were followed up for one year and categorized as stable or progressors based on lung function, deterioration and\/or death. At 18\u201360 months, immunophenotypes were characterized again. (3) The main results showed that: (1) compared to matched controls, at diagnosis, patients with IPF showed more neutrophils, CD8+HLA-DR+ and CD8+CD28\u2212 T cells, and fewer B lymphocytes and na\u00efve T cells; (2) in IPF, circulating neutrophils, eosinophils and na\u00efve T cells were associated with lung function abnormalities; (3) patients whose disease progressed during the 12 months of follow-up showed evidence of cytotoxic dysregulation, with increased CD8+CD28\u2212 T cells, decreased na\u00efve T cells and an inverted CD4\/CD8 ratio at baseline; and (4) blood cell alterations were stable over time in survivors. (4) IPF is associated with abnormalities in circulating immune cells, particularly in the cytotoxic cell domain. Patients with progressive IPF, despite antifibrotic therapy, present an over-activated and exhausted immunophenotype at diagnosis, which is maintained over time.<\/jats:p>","DOI":"10.3390\/ijms241813832","type":"journal-article","created":{"date-parts":[[2023,9,8]],"date-time":"2023-09-08T07:52:11Z","timestamp":1694159531000},"page":"13832","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Blood Immunophenotypes of Idiopathic Pulmonary Fibrosis: Relationship with Disease Severity and Progression"],"prefix":"10.3390","volume":"24","author":[{"given":"Nuria","family":"Mendoza","sequence":"first","affiliation":[{"name":"Institut d\u2019Investigacions Biomediques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), 28029 Madrid, Spain"},{"name":"Biomedicine Department, Universitat de Barcelona, 08036 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Casas-Recasens","sequence":"additional","affiliation":[{"name":"Institut d\u2019Investigacions Biomediques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), 28029 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N\u00faria","family":"Olvera","sequence":"additional","affiliation":[{"name":"Institut d\u2019Investigacions Biomediques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), 28029 Madrid, Spain"},{"name":"Barcelona Supercomputing Center (BSC), 08034 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernanda","family":"Hernandez-Gonzalez","sequence":"additional","affiliation":[{"name":"Institut d\u2019Investigacions Biomediques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"},{"name":"Biomedicine Department, Universitat de Barcelona, 08036 Barcelona, Spain"},{"name":"Respiratory Institute, Clinic Barcelona, 08036 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6362-6348","authenticated-orcid":false,"given":"Tamara","family":"Cruz","sequence":"additional","affiliation":[{"name":"Institut d\u2019Investigacions Biomediques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), 28029 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N\u00faria","family":"Albacar","sequence":"additional","affiliation":[{"name":"Institut d\u2019Investigacions Biomediques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), 28029 Madrid, Spain"},{"name":"Respiratory Institute, Clinic Barcelona, 08036 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5536-6099","authenticated-orcid":false,"given":"Xavier","family":"Alsina-Restoy","sequence":"additional","affiliation":[{"name":"Respiratory Institute, Clinic Barcelona, 08036 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4426-5313","authenticated-orcid":false,"given":"Alejandro","family":"Frino-Garcia","sequence":"additional","affiliation":[{"name":"Respiratory Institute, Clinic Barcelona, 08036 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gemma","family":"L\u00f3pez-Saiz","sequence":"additional","affiliation":[{"name":"Respiratory Institute, Clinic Barcelona, 08036 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas","family":"Robres","sequence":"additional","affiliation":[{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), 28029 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mauricio","family":"Rojas","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alvar","family":"Agust\u00ed","sequence":"additional","affiliation":[{"name":"Institut d\u2019Investigacions Biomediques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES), 28029 Madrid, Spain"},{"name":"Biomedicine Department, Universitat de Barcelona, 08036 Barcelona, Spain"},{"name":"Respiratory Institute, Clinic Barcelona, 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