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Systemic symptoms include fatigue and joint pain and a subset of patients develop more severe disease with multi-organ involvement. Accumulating evidence points to involvement of innate immunity and aberrant activity of the type I IFN system in both the initiation and propagation of this disease. Analysis of the activity of IFN-inducible genes has evidenced that more than half of pSS patients present with a so-called \u2018type I IFN signature\u2019. In this review, we examine activation of the IFN system in pSS patients and how this may drive autoimmunity through various immune cells. We further discuss the clinical value of assessing IFN activity as a biomarker in pSS patients and review novel therapies targeting IFN signalling and their potential use in pSS.<\/jats:p>","DOI":"10.1093\/rheumatology\/key360","type":"journal-article","created":{"date-parts":[[2018,11,12]],"date-time":"2018-11-12T15:07:45Z","timestamp":1542035265000},"page":"2561-2573","source":"Crossref","is-referenced-by-count":82,"title":["Innate immunity and interferons in the pathogenesis of Sj\u00f6gren\u2019s syndrome"],"prefix":"10.1093","volume":"60","author":[{"given":"Iris L A","family":"Bodewes","sequence":"first","affiliation":[{"name":"Department of Immunology, Erasmus University Medical Centre, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albin","family":"Bj\u00f6rk","sequence":"additional","affiliation":[{"name":"Department of Medicine, Rheumatology Unit, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marjan A","family":"Versnel","sequence":"additional","affiliation":[{"name":"Department of Immunology, Erasmus University Medical Centre, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Wahren-Herlenius","sequence":"additional","affiliation":[{"name":"Department of Medicine, Rheumatology Unit, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,2,15]]},"reference":[{"key":"2021061821294799000_key360-B1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/ar2669","article-title":"Epidemiological studies in incidence, prevalence, mortality, and comorbidity of the rheumatic diseases","volume":"11","author":"Gabriel","year":"2009","journal-title":"Arthritis Res Ther"},{"key":"2021061821294799000_key360-B2","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/0049-0172(84)90001-5","article-title":"Primary Sjogren syndrome: clinical and immunopathologic features","volume":"14","author":"Fox","year":"1984","journal-title":"Semin Arthritis Rheum"},{"key":"2021061821294799000_key360-B3","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1046\/j.1365-2796.1996.418817000.x","article-title":"A new model for classification of disease manifestations in primary Sj\u00f6gren\u2019s syndrome: evaluation in a retrospective long-term study","volume":"239","author":"Asmussen","year":"1996","journal-title":"J Internal Med"},{"key":"2021061821294799000_key360-B4","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1098\/rspb.1957.0048","article-title":"Virus interference. 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