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We aimed to systematically evaluate neurological manifestations of primary Sj\u00f6gren\u2019s syndrome and find clinical features allowing sufficient identification of affected patients (pSSN) among those with Sj\u00f6gren\u2019s syndrome without neurological involvement (pSS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Para-\/clinical features of patients with primary Sj\u00f6gren\u2019s syndrome (2016 ACR\/EULAR classification criteria) were compared between pSSN and pSS. At our university-based center, patients with suggestive neurological symptoms undergo screening for Sj\u00f6gren\u2019s syndrome, and newly diagnosed pSS patients are thoroughly evaluated for neurologic involvement. pSSN disease activity was rated by the Neurological Involvement of Sj\u00f6gren\u2019s Syndrome Disease Activity Score (NISSDAI).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      512 patients treated for pSS\/pSSN at our site between 04\/2018 and 07\/2022 were included (238 pSSN patients [46%] vs. 274 pSS patients [54%], cross-sectional design). Independent predictors of neurological involvement in Sj\u00f6gren\u2019s syndrome were male sex [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001], older age at disease onset [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.0001], hospitalization at first presentation [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001], lower IgG levels [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.04] and higher eosinophil values (treatment-na\u00efve) [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02]. Univariate regression additionally showed older age at diagnosis [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001], lower prevalence of rheumatoid factor [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.001], SSA(Ro)\/SSB(La) antibodies [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.03;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001], higher white blood cell count [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02] and CK levels [\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02] (treatment-na\u00efve) in pSSN.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Patients with pSSN had different clinical characteristics than patients with pSS and represented a large proportion of the cohort. Our data suggest that neurological involvement in Sj\u00f6gren\u2019s syndrome has been underestimated. Intensified screening for neurologic involvement should be included in the diagnostic algorithm for Sj\u00f6gren\u2019s syndrome, especially in males of older age and with severe disease course requiring hospitalization.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00415-023-11613-5","type":"journal-article","created":{"date-parts":[[2023,2,19]],"date-time":"2023-02-19T00:15:45Z","timestamp":1676765745000},"page":"2987-2996","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["Sj\u00f6gren\u2019s syndrome with and without neurological involvement"],"prefix":"10.1007","volume":"270","author":[{"given":"Tabea","family":"Seeliger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emelie","family":"Kramer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franz Felix","family":"Konen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadine","family":"Zehrfeld","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonja","family":"Beider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nils Kristian","family":"Prenzler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vega","family":"G\u00f6decke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Torsten","family":"Witte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8550-335X","authenticated-orcid":false,"given":"Thomas","family":"Skripuletz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Ernst","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,2,18]]},"reference":[{"key":"11613_CR1","doi-asserted-by":"publisher","DOI":"10.1111\/ENE.15555","author":"A Liampas","year":"2022","unstructured":"Liampas A, Parperis K, Erotocritou MF et al (2022) Primary Sj\u00f6gren syndrome-related peripheral neuropathy: a systematic review and meta-analysis. 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Outside the submitted work, the authors received honoraria for lectures, travel grants, or research grants: TSe received financial support for conference attendance fees by Abbvie and received honoraria for preparation of a manuscript by Springer. FFK received a travel grant by Merck. NKP received travel grants and congress fees by MED-EL. VG received speaker and travel grants by UCB Pharma, Roche Pharma and Otsuka and participated in advisory boards for Pfizer and Novartis. TW received honoraria for lectures by Abbvie, BMS, Chugai, Galapagos, Janssen, Lilly, Pfizer, UCB, and Roche. DE received presentation honoraria from Abbvie, Amgen, BMS, Chugai, Cilag-Janssen, Galapagos, GSK, Medac, Lilly, Pfizer, Novartis, and Roche and participated in advisory boards for Abbvie, Galapagos, Amgen and Novartis. TSk received honoraria for lectures and participated in advisory boards for Alexion, Alnylam Pharmaceuticals, Bayer Vital, Biogen, Celgene, Centogene, CSL Behring, Euroimmun, Janssen, Merck Serono, Novartis, Roche, Sanofi Aventis, Siemens, Sobi, and Teva. TSe was funded by the Ellen Schmidt Scholarship of the Hannover Medical School. EK was funded by the Else Kr\u00f6ner Fresenius Foundation. NZ was funded by the Else Kr\u00f6ner Fresenius Foundation. TSk received research grants by Alnylam Pharmaceuticals, Bristol-Myers Squibb Foundation for Immuno-Oncology, Claudia von Schilling Foundation, CSL Behring, Else Kr\u00f6ner Fresenius Foundation, Novartis, Sanofi Aventis, VHV Stiftung. SB has nothing to disclose.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"The study was conducted in accordance with the Declaration of Helsinki and approved by the Local Ethics Committee) of the Hannover Medical School (No. 8172_BO_K_2018 and 8179_BO_S_2018).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical standard statement"}}]}}