{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T21:31:55Z","timestamp":1791322315764,"version":"4.3.1"},"reference-count":45,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2005,4,7]],"date-time":"2005-04-07T00:00:00Z","timestamp":1112832000000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Swedish Rheumatism Foundation"},{"name":"Agnes and Mac Rudbergs Foundation"},{"name":"King Gustaf V 80-Year Foundation"},{"DOI":"10.13039\/100007435","name":"\u00c5ke Wiberg Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100007435","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Nanna Svartz Foundation"},{"DOI":"10.13039\/501100006285","name":"Magnus Bergvall Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100006285","id-type":"DOI","asserted-by":"crossref"}]},{"name":"B\u00f6rje Dahlin Foundation"},{"name":"Brunnberg Foundation"},{"name":"Astrid Karlsson Foundation"},{"name":"Uppsala University Hospital Research and Development Fund"},{"name":"Stifud Foundation"},{"name":"Groschinsky Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatism"],"published-print":{"date-parts":[[2005,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The etiopathogenesis of primary Sj\u00f6gren's syndrome (SS) is largely unknown. In other autoimmune diseases, type I interferon (IFN) may play a pivotal role by triggering and sustaining the disease process. We therefore aimed to determine whether patients with primary SS had an activated type I IFN system.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Salivary gland biopsy specimens and sera from patients with primary SS were investigated for the occurrence of IFN\u03b1\u2010producing cells and measurable IFN\u03b1 levels, respectively. The ability of primary SS sera together with apoptotic or necrotic cells to induce IFN\u03b1 production in normal peripheral blood mononuclear cells was examined. The IFN\u03b1 inducer was characterized, and IFN\u03b1\u2010producing cells were identified. Clinical data were correlated with the IFN\u03b1\u2010inducing capacity of primary SS sera.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Numerous IFN\u03b1\u2010producing cells were detected in salivary gland biopsy specimens, despite low serum IFN\u03b1 levels. Autoantibodies to RNA\u2010binding proteins, combined with material released by necrotic or late apoptotic cells, were potent inducers of IFN\u03b1 production in plasmacytoid dendritic cells (PDCs). This appeared to be attributable to RNA\u2010containing immune complexes triggering PDCs by means of RNA and interaction with Fc\u03b3 receptor IIa. The IFN\u03b1\u2010inducing capacity of sera was associated with positive results of a labial salivary gland biopsy (focus score \u22651) and with dermatologic, hematologic, and pulmonary manifestations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Patients with primary SS have an activated type I IFN system. Although virus may initiate the production of IFN, the continued IFN\u03b1 synthesis is caused by RNA\u2010containing immune complexes that activate PDCs to prolong IFN\u03b1 production at the tissue level. This IFN\u03b1 promotes the autoimmune process by a vicious circle\u2013like mechanism, with increased autoantibody production and formation of more endogenous IFN\u03b1 inducers.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.20998","type":"journal-article","created":{"date-parts":[[2005,4,7]],"date-time":"2005-04-07T11:23:00Z","timestamp":1112872980000},"page":"1185-1195","source":"Crossref","is-referenced-by-count":350,"title":["Activation of the type I interferon system in primary Sj\u00f6gren's syndrome: A possible etiopathogenic 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