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Women are more prone to develop pSS than men, with a sex ratio of 9:1. Considered in the past as innocent collateral passive victims of autoimmunity, the epithelial cells of the salivary glands are now known to play an active role in the pathogenesis of the disease. The aetiology of the \u201cautoimmune epithelitis\u201d still remains unknown, but certainly involves genetic, environmental and hormonal factors. Later during the disease evolution, the subsequent chronic activation of B cells can lead to the development of systemic manifestations or non-Hodgkin\u2019s lymphoma. The aim of the present comprehensive review is to provide the current state of knowledge on pSS. The review addresses the clinical manifestations and complications of the disease, the diagnostic workup, the pathogenic mechanisms and the therapeutic approaches.<\/jats:p>","DOI":"10.3390\/jcm9072299","type":"journal-article","created":{"date-parts":[[2020,7,22]],"date-time":"2020-07-22T03:23:16Z","timestamp":1595388196000},"page":"2299","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":166,"title":["Current State of Knowledge on Primary Sj\u00f6gren\u2019s Syndrome, an Autoimmune Exocrinopathy"],"prefix":"10.3390","volume":"9","author":[{"given":"Dorian","family":"Parisis","sequence":"first","affiliation":[{"name":"Laboratory of Pathophysiological and Nutritional Biochemistry, Universit\u00e9 Libre de Bruxelles, 1070 Brussels, Belgium"},{"name":"Department of Rheumatology, Erasme Hospital, Universit\u00e9 Libre de Bruxelles, 1070 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara","family":"Chivasso","sequence":"additional","affiliation":[{"name":"Laboratory of Pathophysiological and Nutritional Biochemistry, Universit\u00e9 Libre de Bruxelles, 1070 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason","family":"Perret","sequence":"additional","affiliation":[{"name":"Laboratory of Pathophysiological and Nutritional Biochemistry, Universit\u00e9 Libre de Bruxelles, 1070 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad Shahnawaz","family":"Soyfoo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Erasme Hospital, Universit\u00e9 Libre de Bruxelles, 1070 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0385-5824","authenticated-orcid":false,"given":"Christine","family":"Delporte","sequence":"additional","affiliation":[{"name":"Laboratory of Pathophysiological and Nutritional Biochemistry, Universit\u00e9 Libre de Bruxelles, 1070 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,7,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/S0140-6736(05)66990-5","article-title":"Sj\u00f6gren\u2019s syndrome","volume":"366","author":"Fox","year":"2005","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Gerli, R., Bartoloni, E., and Alunno, A. 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