{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T17:12:18Z","timestamp":1790874738921,"version":"4.1.0"},"reference-count":11,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2023,5,9]],"date-time":"2023-05-09T00:00:00Z","timestamp":1683590400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["1K23\u2010AR\u2010075898"],"award-info":[{"award-number":["1K23\u2010AR\u2010075898"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["K08\u2010AR\u2010077732"],"award-info":[{"award-number":["K08\u2010AR\u2010077732"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["P30\u2010AR\u2010070254"],"award-info":[{"award-number":["P30\u2010AR\u2010070254"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["R01\u2010AR\u2010073208"],"award-info":[{"award-number":["R01\u2010AR\u2010073208"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To describe the disease specificity, clinical phenotype, and risk of cancer in dermatomyositis (DM) patients with autoantibodies against cell division cycle and apoptosis regulator protein 1 (anti\u2010CCAR1).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The frequency of anti\u2010CCAR1 autoantibodies was measured by enzyme\u2010linked immunosorbent assay in the serum of DM patients from 2 independent cohorts (Johns Hopkins and Stanford), with patients with several other rheumatic diseases and healthy controls used as comparators. Clinical features and the risk of cancer incidence relative to that in the general population were determined in anti\u2010CCAR1\u2013positive DM patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Anti\u2010CCAR1 antibodies were significantly associated with anti\u2013transcriptional intermediary factor 1\u03b3 (anti\u2010TIF1\u03b3) antibodies present in the serum of patients with DM: 80 (32%) of 252 anti\u2010TIF1\u03b3\u2013positive DM patients versus 14 (8%) of 186 anti\u2010TIF1\u03b3\u2013negative DM patients were positive for anti\u2010CCAR1 antibodies (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). Anti\u2010CCAR1 antibodies were not detected in any of the 32 serum samples from healthy controls, and were present at very low frequencies in the sera of patients with other rheumatic diseases: 1 (2.3%) of 44 patients with anti\u2013hydroxymethylglutaryl\u2010coenzyme A reductase\u2013positive necrotizing myopathy, 1 (2.3%) of 44 patients with inclusion body myositis, and 3 (6.5%) of 46 patients with systemic lupus erythematosus were positive for anti\u2010CCAR1 antibodies. Upon examining data on occurrence of cancer from the onset of DM onward, the observed number of cancers diagnosed in anti\u2013TIF\u20101\u03b3\u2013positive DM patients was significantly greater than expected in both cohorts, with a standardized incidence ratio (SIR) of 3.49 (95% confidence interval [95% CI] 2.39\u20134.92) in the Johns Hopkins cohort and a SIR of 4.54 (95% CI 3.04\u20136.52) in the Stanford cohort (each <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). DM patients who were both anti\u2010TIF1\u03b3 positive and anti\u2010CCAR1 positive had lower SIRs for cancer, with a SIR of 1.78 (95% CI 0.77\u20133.51) (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.172) in the Johns Hopkins cohort and a SIR of 1.61 (95% CI 0.44\u20134.13) (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.48) in the Stanford cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Anti\u2010CCAR1 autoantibodies are specific for anti\u2010TIF1\u03b3\u2013positive DM. Their presence in anti\u2010TIF1\u03b3\u2013positive patients attenuates the risk of cancer to a level comparable to that seen in the general population.<\/jats:p><jats:p><jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/art42474-toc-0001-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text><\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42474","type":"journal-article","created":{"date-parts":[[2023,2,10]],"date-time":"2023-02-10T04:02:25Z","timestamp":1676001745000},"page":"1238-1245","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["Association of <scp>Anti\u2010CCAR1<\/scp> Autoantibodies With Decreased Cancer Risk Relative to the General Population in Patients With Anti\u2013Transcriptional Intermediary Factor 1\u03b3\u2013Positive Dermatomyositis"],"prefix":"10.1002","volume":"75","author":[{"given":"David","family":"Fiorentino","sequence":"first","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine  Redwood City California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1429-8392","authenticated-orcid":false,"given":"Christopher A.","family":"Mecoli","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tak","family":"Igusa","sequence":"additional","affiliation":[{"name":"Whiting School of Engineering Johns Hopkins University  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8030-9504","authenticated-orcid":false,"given":"Jemima","family":"Albayda","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8436-1601","authenticated-orcid":false,"given":"Julie J.","family":"Paik","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4749-870X","authenticated-orcid":false,"given":"Eleni","family":"Tiniakou","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brittany","family":"Adler","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3732-3252","authenticated-orcid":false,"given":"Andrew L.","family":"Mammen","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine, Baltimore, Maryland, and Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ami A.","family":"Shah","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology Johns Hopkins University School of Medicine  Baltimore 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SEER*Stat Database: Incidence\u2014SEER Research Data 9 Registries Nov 2020 Sub (1975\u20132018)\u2014Linked To County Attributes\u2014Time Dependent (1990\u20132018) Income\/Rurality 1969\u20132019 Counties. 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