{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T21:12:31Z","timestamp":1760044351649},"reference-count":40,"publisher":"Hogrefe Publishing Group","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Praxis"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:p> Zusammenfassung. Das prim\u00e4re Sj\u00f6gren-Syndrom ist eine autoimmune Erkrankung vornehmlich der exokrinen Dr\u00fcsen und \u00e4ussert sich meistens in Sicca-Symptomatik der Augen und des Mundes, jedoch auch von Nase, Rachen, Vagina und Haut. Fatigue und Schmerz sind ebenfalls sehr charakteristisch. Die Erkrankung kann sich jedoch auch systemisch manifestieren, z.B. in Gelenken, Muskeln, Lungen, Nieren, Haut und Nervensystem. Gef\u00fcrchtet ist die Entwicklung eines Lymphoms, das insbesondere bei Vorliegen von Anti-SSA-(Ro)-positiven Antik\u00f6rpern, aber auch z.B. bei hohem ESSDAI-Wert, Komplementverbrauch, Zytopenien oder Nachweis von ektopen Keimzentren in der Speicheldr\u00fcsenbiopsie erh\u00f6ht ist. Die Diagnosestellung ergibt sich in der Regel durch Objektivierung der Sicca-Symptomatik (z.B. Schirmertest) und Vorhandensein der typischen Antik\u00f6rper oder bei typischer Speicheldr\u00fcsenbiopsie. Therapeutisch wird die Sicca-Symptomatik prim\u00e4r symptomatisch behandelt (z.B. durch Tr\u00e4nen- und Speichelersatzmittel), erst bei systemischen Manifestationen kommen Glukokortikoide, konventionelle DMARDs oder ggf. Biologika (in der Regel Rituximab) zum Einsatz. Bei sehr schweren Verl\u00e4ufen sind ggf. zus\u00e4tzlich intraven\u00f6se Immunglobuline und Plasmaexchange notwendig. <\/jats:p>","DOI":"10.1024\/1661-8157\/a003442","type":"journal-article","created":{"date-parts":[[2020,4,28]],"date-time":"2020-04-28T21:14:01Z","timestamp":1588108441000},"page":"333-339","source":"Crossref","is-referenced-by-count":4,"title":["Prim\u00e4res Sj\u00f6gren-Syndrom \u2013 Neues zu Diagnostik                     und Therapie"],"prefix":"10.1024","volume":"109","author":[{"given":"Marco","family":"Sprecher","sequence":"first","affiliation":[{"name":"Klinik f\u00fcr Rheumatologie, Universit\u00e4tsspital                     Z\u00fcrich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Britta","family":"Maurer","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Rheumatologie, Universit\u00e4tsspital                     Z\u00fcrich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oliver","family":"Distler","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Rheumatologie, Universit\u00e4tsspital                     Z\u00fcrich"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"101","reference":[{"key":"c1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.03.008"},{"key":"c2","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-209952"},{"key":"c3","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kev420"},{"key":"c4","doi-asserted-by":"publisher","DOI":"10.1002\/art.39859"},{"key":"c5","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-017-3718-1"},{"key":"c6","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-3083.2011.02610.x"},{"key":"c7","doi-asserted-by":"publisher","DOI":"10.1191\/0961203302lu252oa"},{"key":"c8","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-019-04438-z"},{"key":"c9","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2013.278448"},{"key":"c10","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.e3821"},{"key":"c11","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ket349"},{"key":"c12","unstructured":"Borg EJ,                                 Kelder JC:                         Lower prevalence of extra-glandular manifestations and                         anti-SSB antibodies in patients with primary Sj\u00f6gren\u2019s                         syndrome and widespread pain: evidence for a relatively benign                         subset. 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