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APECED is an inborn error of immunity characterized by immune dysregulation and chronic mucocutaneous candidiasis. Reduction in the frequency of circulating MAITs has been reported in many inborn errors of immunity, but only in a few of them, the functional competence of MAITs has been assessed. Here, we show in a cohort of 24 patients with APECED, that the proportion of circulating MAITs was reduced compared with healthy age and sex\u2010matched controls (1.1% vs. 2.6% of CD3\n                    <jats:sup>+<\/jats:sup>\n                    T cells;\n                    <jats:italic>p<\/jats:italic>\n                    &lt; 0.001) and the MAIT cell immunophenotype was more activated. Functionally the IFN\u2010\u03b3 secretion of patient MAITs after stimulation was comparable to healthy controls. We observed in the patients elevated serum IFN\u2010\u03b3 (46.0 vs. 21.1 pg\/mL;\n                    <jats:italic>p<\/jats:italic>\n                    = 0.01) and IL\u201018 (42.6 vs. 13.7 pg\/mL;\n                    <jats:italic>p<\/jats:italic>\n                    &lt; 0.001) concentrations. Lower MAIT proportion did not associate with the levels of neutralizing anti\u2010IL\u201022 or anti\u2010IL\u201012\/23 antibodies but had a clear negative correlation with serum concentrations of IFN\u2010\u03b3, IL\u201018, and protein C\u2010reactive protein. Our data suggest that reduction of circulating MAITs in patients with APECED correlates with chronic type 1 inflammation but the remaining MAITs are functionally competent.\n                  <\/jats:p>","DOI":"10.1002\/eji.202451189","type":"journal-article","created":{"date-parts":[[2024,9,18]],"date-time":"2024-09-18T10:41:04Z","timestamp":1726656064000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Reduction in mucosal\u2010associated invariant T cells (MAIT) in APECED patients is associated with elevated serum IFN\u2010\u03b3 concentration"],"prefix":"10.1002","volume":"54","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6576-3549","authenticated-orcid":false,"given":"Iivo","family":"Hetem\u00e4ki","sequence":"first","affiliation":[{"name":"Translational Immunology Research Program University of Helsinki and Helsinki University Hospital  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joona","family":"Sarkkinen","sequence":"additional","affiliation":[{"name":"Translational Immunology Research Program University of Helsinki and Helsinki University Hospital  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huai Hui","family":"Wong","sequence":"additional","affiliation":[{"name":"ImmuDocs National Doctoral Education Pilot Program University of Helsinki  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8879-1569","authenticated-orcid":false,"given":"Nelli","family":"Heikkil\u00e4","sequence":"additional","affiliation":[{"name":"Translational Immunology Research Program University of Helsinki and Helsinki University Hospital  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saila","family":"Laakso","sequence":"additional","affiliation":[{"name":"Children's Hospital University of Helsinki and Helsinki University Hospital  Helsinki Finland"},{"name":"Research Program for Clinical and Molecular Metabolism, Faculty of Medicine University of Helsinki  Helsinki Finland"},{"name":"Folkh\u00e4lsan Research Center Institute of Genetics  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simo","family":"Miettinen","sequence":"additional","affiliation":[{"name":"Translational Immunology Research Program University of Helsinki and Helsinki University Hospital  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mikko I.","family":"M\u00e4yr\u00e4np\u00e4\u00e4","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Helsinki and Helsinki University Hospital  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Outi","family":"M\u00e4kitie","sequence":"additional","affiliation":[{"name":"Children's Hospital University of Helsinki and Helsinki University Hospital  Helsinki Finland"},{"name":"Research Program for Clinical and Molecular Metabolism, Faculty of Medicine University of Helsinki  Helsinki Finland"},{"name":"Folkh\u00e4lsan Research Center Institute of Genetics  Helsinki Finland"},{"name":"Department of Molecular Medicine, Karolinska Institutet, and Clinical Genetics Karolinska University Hospital  Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T Petteri","family":"Arstila","sequence":"additional","affiliation":[{"name":"Translational Immunology Research Program University of Helsinki and Helsinki University Hospital  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eliisa","family":"Kek\u00e4l\u00e4inen","sequence":"additional","affiliation":[{"name":"Translational Immunology Research Program University of Helsinki and Helsinki University Hospital  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,9,18]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri.2016.9"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2021.723532"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2021.108851"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20210554"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20091983"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20091669"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.aay5731"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2024.02.012"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2020.09.009"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coi.2023.102351"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms11653"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.29782"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/eji.201343509"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41590-019-0444-8"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.00838"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.1107609"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1812273115"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature13160"},{"key":"e_1_2_11_20_1","article-title":"Fecal 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Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)\u2010Associated Alopecia Areata. Clinical Trial2023."}],"container-title":["European Journal of Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/eji.202451189","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,6]],"date-time":"2025-06-06T00:57:47Z","timestamp":1749171467000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/eji.202451189"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,18]]},"references-count":34,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2024,12]]}},"alternative-id":["10.1002\/eji.202451189"],"URL":"https:\/\/doi.org\/10.1002\/eji.202451189","archive":["Portico"],"relation":{"has-review":[{"id-type":"doi","id":"10.1002\/EJI.202451189\/v1\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v3\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v2\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v2\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v1\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v1\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v2\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v2\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/EJI.202451189\/v3\/decision1","asserted-by":"object"}]},"ISSN":["0014-2980","1521-4141"],"issn-type":[{"value":"0014-2980","type":"print"},{"value":"1521-4141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,18]]},"assertion":[{"value":"2024-04-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-09-05","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-09-18","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"2451189"}}