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The aims of the present study were to examine whether these autoantibodies occur in patients with juvenile\u2010onset DM (JDM) and to determine their associated features.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Sera from 150 patients with anti\u2010TIF1\u03b3 autoantibody\u2010positive JDM in a cross\u2010sectional cohort and 90 juvenile healthy controls were assayed for anti\u2010CCAR1, anti\u2010C1Z1, anti\u2010IMMT, anti\u2010TBL1XR1, and anti\u2010Sp4 autoantibodies. Demographics, myositis autoantibodies, clinical features, medications, outcomes, and <jats:italic>HLA\u2010DRB1<\/jats:italic> and <jats:italic>HLA\u2010DQA1<\/jats:italic> alleles were compared between those with and without these autoantibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Any one of the anti\u2010TIF1\u03b3\u2010associated autoantibodies was present in 44 patients (29%) overall, including 25 (17%) with anti\u2010Sp4, 22 (15%) with anti\u2010TBL1XR1, 14 (9%) with anti\u2010CCAR1, 2 (1%) with anti\u2010C1Z1, and 2 (1%) with anti\u2010IMMT autoantibodies. These anti\u2010TIF1\u03b3\u2010associated autoantibodies frequently co\u2010occurred. Patients with any of the anti\u2010TIF1\u03b3\u2010associated autoantibodies had less frequent falling (34% [15] vs. 53% [56], <jats:italic>P<\/jats:italic> =\u20090.032) and lower peak muscle enzymes. None of the patients had cancer. Among White patients, HLA\u2010DRB1*03 was protective against an anti\u2010TIF1\u03b3\u2010associated autoantibody (odds ratio 0.20, 95% confidence interval 0.07\u20130.52).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Autoantibodies associated with anti\u2010TIF1\u03b3 were found in isolation and in combination among a subset of patients with JDM. Patients with these autoantibodies had less severe muscle disease and were not enriched for <jats:italic>HLA\u2010DRB1*03<\/jats:italic>. Additional autoantibodies among patients with positive anti\u2010TIF1\u03b3 with JDM likely contribute to the heterogeneity of the anti\u2010TIF1\u03b3 serologic subgroup.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42768","type":"journal-article","created":{"date-parts":[[2023,12,7]],"date-time":"2023-12-07T19:13:35Z","timestamp":1701976415000},"page":"631-637","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Clinical Features and Immunogenetic Risk Factors Associated With Additional Autoantibodies in Anti\u2010Transcriptional Intermediary Factor 1\u03b3 <scp>Juvenile\u2010Onset<\/scp> Dermatomyositis"],"prefix":"10.1002","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5448-1538","authenticated-orcid":false,"given":"Matthew A.","family":"Sherman","sequence":"first","affiliation":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases National Institutes of Health  Bethesda 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