{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T19:40:39Z","timestamp":1789501239819,"version":"build-2803163510"},"reference-count":32,"publisher":"BMJ","issue":"1","license":[{"start":{"date-parts":[[2023,11,23]],"date-time":"2023-11-23T00:00:00Z","timestamp":1700697600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/100020799","name":"Association des Sclerodermiques de France","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100020799","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Scleroderma and Related Disorders"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>Systemic sclerosis is an autoimmune disease characterized by fibrosis of the skin and internal organs including the lung. Mucosal-associated invariant T cells are innate-like T lymphocytes able to produce various cytokines and cytotoxic mediators such as granzyme B. A large body of evidence supports a role of mucosal-associated invariant T cells in autoimmune disease but more recent reports suggest also a potential role in fibrotic conditions. Therefore, we herein addressed the question as whether mucosal-associated invariant T cells may have an altered profile in systemic sclerosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Mucosal-associated invariant T cell frequency was analyzed by flow cytometry, using fresh peripheral blood from 74 consecutive systemic sclerosis patients who were compared to 44 healthy donors. In addition, in-depth mucosal-associated invariant T cell phenotype and function were analyzed in unselected 29 women with systemic sclerosis who were compared to 23 healthy women donors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Proportion of circulating mucosal-associated invariant T cells was significantly reduced by 68% in systemic sclerosis compared to healthy donors (0.78% in systemic sclerosis vs 2.5%, p &lt; 0.0001). Within systemic sclerosis subsets, mucosal-associated invariant T cells were reduced in patients with interstitial lung disease (systemic sclerosis-interstitial lung disease) (0.56% vs 0.96% in patients without interstitial lung disease, p = 0.04). Moreover, in systemic sclerosis patients, mucosal-associated invariant T cells displayed an activated phenotype indicated by markedly increased CD69\n                      <jats:sup>+<\/jats:sup>\n                      mucosal-associated invariant T cell frequency (20% mucosal-associated invariant T cell CD69\n                      <jats:sup>+<\/jats:sup>\n                      compared to 9.4% in healthy donors, p = 0.0014). Interestingly, mucosal-associated invariant T cells from systemic sclerosis-interstitial lung disease patients had a more pronounced altered phenotype compared to systemic sclerosis without interstitial lung disease with a correlation between mucosal-associated invariant T cells expressing CCR6\n                      <jats:sup>+<\/jats:sup>\n                      and mucosal-associated invariant T cell frequency (r = 0.8, p = 0.006).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>Circulating mucosal-associated invariant T cells were reduced and exhibited an activated phenotype in systemic sclerosis patients. This peripheral mucosal-associated invariant T cell deficiency may be related to enhanced apoptosis and\/or homing in inflamed tissue, particularly in systemic sclerosis-interstitial lung disease patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/23971983231209807","type":"journal-article","created":{"date-parts":[[2023,11,24]],"date-time":"2023-11-24T02:05:22Z","timestamp":1700791522000},"page":"67-78","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":3,"title":["Deficiency and altered phenotype of mucosal-associated invariant T cells in systemic sclerosis"],"prefix":"10.1136","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7794-2096","authenticated-orcid":false,"given":"Manon","family":"Lesturgie-Talarek","sequence":"first","affiliation":[{"name":"Institut Cochin, INSERM U1016, UMR 8104, Paris, 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France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2023,11,23]]},"reference":[{"key":"bibr1-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1177\/2397198320924873"},{"key":"bibr2-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2015.2"},{"key":"bibr3-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kes090"},{"key":"bibr4-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1177\/23971983211039421"},{"key":"bibr5-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1002\/art.37860"},{"key":"bibr6-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1006\/clin.1996.4322"},{"key":"bibr7-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1111\/imr.12423"},{"key":"bibr8-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1038\/nature01433"},{"key":"bibr9-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1002\/eji.201343509"},{"key":"bibr10-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1003681"},{"key":"bibr11-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1038\/mi.2014.81"},{"key":"bibr12-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1111\/imcb.12011"},{"key":"bibr13-23971983231209807","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2018.01333"},{"key":"bibr14-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-018-04450-y"},{"key":"bibr15-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1111\/sji.12585"},{"key":"bibr16-23971983231209807","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2013-204424"},{"key":"bibr17-23971983231209807","doi-asserted-by":"publisher","DOI":"10.5301\/jsrd.5000231"},{"key":"bibr18-23971983231209807","volume":"21","author":"Medsger 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