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Myositis\u2010specific and \u2010associated autoantibodies were determined by Euroimmun line blot, enzyme\u2010linked immunosorbent assay, and immunoprecipitation. We calculated standardized prevalence ratios (SPRs) and adjusted for calendar year, age, sex, race, and ethnicity by comparing observed cancers in IIM patients versus expected cancers in the general population using the Surveillance, Epidemiology, and End Results registry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 1,172 IIM patients, 203 (17%) patients with a cancer history were studied. Over a median follow\u2010up of 5.2\u2009years, the observed number of IIM patients diagnosed with cancer was increased 1.43\u2010fold (SPR 1.43 [95% confidence interval (95% CI) 1.15\u20131.77]; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.002). Within 3\u2009years of IIM symptom onset, an increased SPR was observed for anti\u2013transcription intermediary factor 1\u03b3 (anti\u2010TIF1\u03b3)\u2013positive patients for ovarian and breast cancer (ovarian SPR 18.39 [95% CI 5.01\u201347.08], <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001; breast SPR 3.84 [95% CI 1.99\u20136.71], <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). As expected, anti\u2010TIF1\u03b3 positivity was associated with a significantly elevated SPR; however, only 55% (36 of 66) of all cancers within 3\u2009years of dermatomyositis onset were observed in anti\u2010TIF1\u03b3\u2013positive patients. Other myositis\u2010specific autoantibodies, including anti\u2013Mi\u20102, anti\u2013small ubiquitin\u2010like modifier activating enzyme (SAE), and anti\u2010nuclear matrix protein 2 (NXP\u20102), accounted for 26% (17 of 66) of cancers diagnosed within 3\u2009years of dermatomyositis onset. No cancer association, positive or negative, was observed for patients with antisynthetase, anti\u2013melanoma differentiation\u2013associated protein 5 (anti\u2013MDA\u20105), or anti\u2013hydroxymethylglutaryl\u2010coenzyme A reductase (anti\u2010HMGCR) antibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In a tertiary referral center population, anti\u2010TIF1\u03b3 was most strongly associated with breast and ovarian cancer. Patients with antisynthetase, anti\u2013MDA\u20105, or anti\u2010HMGCR antibodies had the same cancer risk as 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\/art42311-toc-0001-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text><\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42311","type":"journal-article","created":{"date-parts":[[2022,7,25]],"date-time":"2022-07-25T15:19:40Z","timestamp":1658762380000},"page":"620-629","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":48,"title":["Subsets of Idiopathic Inflammatory Myositis Enriched for Contemporaneous Cancer Relative to the General 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