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We here investigated whether infections increase the risk of developing primary Sj\u00f6gren's syndrome (<jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content> in Sweden (<jats:italic>n<\/jats:italic>\u00a0=\u00a0945) and matched controls from the general population (<jats:italic>n<\/jats:italic>\u00a0=\u00a09048) were included, and data extracted from the National Patient Register to identify infections occurring before <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content> diagnosis during a mean observational time of 16.0\u00a0years. Data were analysed using conditional logistic regression models. Sensitivity analyses were performed by varying exposure definition and adjusting for previous health care consumption.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A history of infection associated with an increased risk of <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 1.9, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.6\u20132.3). Infections were more prominently associated with the development of <jats:styled-content style=\"fixed-case\">SSA<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">SSB<\/jats:styled-content> autoantibody\u2010positive <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 2.7, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 2.0\u20133.5). When stratifying the analysis by organ system infected, respiratory infections increased the risk of developing <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content>, both in patients with (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 2.9, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.8\u20134.7) and without autoantibodies (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 2.1, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.1\u20133.8), whilst skin and urogenital infections only significantly associated with the development of autoantibody\u2010positive <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 3.2, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.8\u20135.5 and <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> 2.7, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.7\u20134.2). Furthermore, a dose\u2013response relationship was observed for infections and a risk to develop <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content> with Ro\/<jats:styled-content style=\"fixed-case\">SSA<\/jats:styled-content> and La\/<jats:styled-content style=\"fixed-case\">SSB<\/jats:styled-content> antibodies. Gastrointestinal infections were not significantly associated with a risk of <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Infections increase the risk of developing <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content>, most prominently <jats:styled-content style=\"fixed-case\">SSA<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">SSB<\/jats:styled-content> autoantibody\u2010positive disease, suggesting that microbial triggers of immunity may partake in the pathogenetic process of <jats:styled-content style=\"fixed-case\">pSS<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/joim.12888","type":"journal-article","created":{"date-parts":[[2019,3,20]],"date-time":"2019-03-20T10:31:00Z","timestamp":1553077860000},"page":"670-680","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Infections increase the risk of developing Sj\u00f6gren's syndrome"],"prefix":"10.1111","volume":"285","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1873-7169","authenticated-orcid":false,"given":"J.","family":"Mofors","sequence":"first","affiliation":[{"name":"Division of Rheumatology Department of Medicine Solna Karolinska Institutet Karolinska University Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. 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