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All participants underwent single\u2010nucleotide polymorphism typing. We examined X and Y single\u2010nucleotide polymorphism heterogeneity to determine the number of X chromosomes. For statistical comparisons, we used \u03c7<jats:sup>2<\/jats:sup> analyses with calculation of 95% confidence intervals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Three of seventy men with SSc had 47,XXY (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.0001 compared with control men). Among the 435 women with SSc, none had 47,XXX. Among 709 men with polymyositis or dermatomyositis (PM\/DM), seven had 47,XXY (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.0016), whereas among the 1783 women with PM\/DM, two had 47,XXX. Of 147 men with inclusion body myositis (IBM), six had 47,XXY, and 1 of the 114 women with IBM had 47,XXX. For each of these myositis disease groups, the excess 47,XXY and\/or 47,XXX was significantly higher compared with in controls as well as the known birth rate of Klinefelter syndrome or 47,XXX.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Klinefelter syndrome (47,XXY) is associated with SSc and idiopathic inflammatory myopathies, similar to other autoimmune diseases with type 1 interferon pathogenesis, namely, systemic lupus erythematosus and Sj\u00f6gren syndrome.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr2.11413","type":"journal-article","created":{"date-parts":[[2022,3,30]],"date-time":"2022-03-30T01:33:30Z","timestamp":1648604010000},"page":"528-533","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["<scp>47XXY<\/scp> and <scp>47XXX<\/scp> in Scleroderma and Myositis"],"prefix":"10.1002","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1015-5850","authenticated-orcid":false,"given":"R. 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Boas Center for Genomics and Human Genetics, Feinstein Institutes for Medical Research  Manhasset New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Lee","sequence":"additional","affiliation":[{"name":"Robert S. Boas Center for Genomics and Human Genetics, Feinstein Institutes for Medical Research  Manhasset New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6912-2458","authenticated-orcid":false,"given":"Lisa G.","family":"Rider","sequence":"additional","affiliation":[{"name":"National Institute of Environmental Health Science, National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terrance","family":"O'Hanlon","sequence":"additional","affiliation":[{"name":"National Institute of Environmental Health Science, National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Rothwell","sequence":"additional","affiliation":[{"name":"The University of Manchester  Manchester 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