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Research"],"published-print":{"date-parts":[[2020,8]]},"abstract":"Objective<\/jats:title>To describe the clinical and serologic manifestations of Sj\u00f6gren's syndrome (SS<\/jats:styled-content>) in ethnic groups of the US<\/jats:styled-content>.<\/jats:p><\/jats:sec>Methods<\/jats:title>This was a cross\u2010sectional study of 648 patients with primary SS<\/jats:styled-content>: 20 African American (AA<\/jats:styled-content>), 164 American Indian (AI<\/jats:styled-content>), 426 European American (EA<\/jats:styled-content>), and 38 patients of other races evaluated in a multidisciplinary Sj\u00f6gren\u2019s International Collaborative Clinical Alliance research clinic.<\/jats:p><\/jats:sec>Results<\/jats:title>AA<\/jats:styled-content> subjects comprised 3.1% of the SS<\/jats:styled-content> cohort, much lower than the percentage of AA<\/jats:styled-content> in the state of Oklahoma (P<\/jats:italic> = 3.01 \u00d7 E \u2013 05), the US<\/jats:styled-content> (P<\/jats:italic> = 2.24E \u2013 13), or a systemic lupus erythematosus (SLE<\/jats:styled-content>) cohort at the same institution (P<\/jats:italic> = 4.26 \u00d7 10E \u2013 27). In contrast, the percentage of AI<\/jats:styled-content> in the SS<\/jats:styled-content> cohort (25.3%) was much higher than expected (P<\/jats:italic> = 3.17E \u2013 09 versus SLE<\/jats:styled-content> cohort, P<\/jats:italic> = 6.36 \u2013 26 versus Oklahoma, and P<\/jats:italic> = 8.14E \u2013 96 versus US<\/jats:styled-content> population). The SS<\/jats:styled-content> classification criteria were similar between AA<\/jats:styled-content> and EA<\/jats:styled-content>, but subjects of AI<\/jats:styled-content> ancestry had lower rates of abnormal tear and salivary flow, as well as anti\u2010Ro\/SSA<\/jats:styled-content> and anti\u2010La\/SSB<\/jats:styled-content> antibodies. Paradoxically, AI<\/jats:styled-content> had higher levels of disease activity (mean \u00b1 SD<\/jats:styled-content> European League Against Rheumatism Sj\u00f6gren's Syndrome Disease Activity Index score 3.77 \u00b1 4.78) in comparison to EA<\/jats:styled-content> (2.90 \u00b1 4.12; P<\/jats:italic> = 0.011) and more extraglandular manifestations affecting mainly the articular and glandular domains. Meanwhile, AA<\/jats:styled-content> patients were characterized by higher rates of hypergammaglobulinemia (odds ratio [OR<\/jats:styled-content>] 1.39 [95% confidence interval (95% CI<\/jats:styled-content>) 1.39\u20138.65]; P<\/jats:italic> = 0.01), elevated erythrocyte sedimentation rate (ESR<\/jats:styled-content>) (OR<\/jats:styled-content> 3.95 [95% CI<\/jats:styled-content> 1.46\u20139.95]; P<\/jats:italic> = 0.009), and parotid enlargement (OR<\/jats:styled-content> 4.40 [95% CI<\/jats:styled-content> 1.49\u201313.07]; P<\/jats:italic> = 0.02).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>AI<\/jats:styled-content> are affected at high rates with SS<\/jats:styled-content> but present with few classical features, potentially preventing timely diagnosis. In contrast to SLE<\/jats:styled-content>, SS<\/jats:styled-content> is infrequent and not more severe among AA<\/jats:styled-content>, but the triad of hypergammaglobulinemia, increased ESR<\/jats:styled-content>, and parotid enlargement warrants extra vigilance for lymphomagenesis.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.24003","type":"journal-article","created":{"date-parts":[[2019,6,14]],"date-time":"2019-06-14T11:09:10Z","timestamp":1560510550000},"page":"1049-1056","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["American Indians Have a Higher Risk of Sj\u00f6gren's Syndrome and More Disease Activity Than European Americans and African Americans"],"prefix":"10.1002","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1015-5850","authenticated-orcid":false,"given":"R. 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