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We therefore aimed to characterize patients with SFN and Sj\u00f6gren\u2019s syndrome to raise awareness of this disease and facilitate its early recognition as an essential step for appropriate treatment. In 97 SFN patients (median age 48\u00a0years, 77% female), we studied the clinical features associated with Sj\u00f6gren\u2019s syndrome compared to the idiopathic SFN subtype. According to the current ACR\/EULAR classification criteria (Shiboski et al., Ann Rheum Dis 76:9\u201316, 2017), 24\/97 individuals (25%, median age 48.5\u00a0years, 75% female) were diagnosed with Sj\u00f6gren\u2019s syndrome. We did not observe any differences in SFN-defining sensory plus symptoms. Furthermore, intraepidermal nerve fiber densities (IENFD) were significantly lower in patients with SFN and Sj\u00f6gren\u2019s syndrome (mean 2.6\u2009\u00b1\u20091.2\/mm) compared to patients with idiopathic SFN (mean 3.2\u2009\u00b1\u20091.5\/mm;\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.048). There were no significant group differences when analyzing cerebrospinal fluid (CSF) parameters. We conclude that Sj\u00f6gren\u2019s syndrome-associated SFN is difficult to distinguish from idiopathic forms based on initial clinical symptoms and CSF results. However, lower IENFD values in patients with Sj\u00f6gren's syndrome-associated SFN might indicate a distinct different pathomechanism in this entity compared to idiopathic SFN.\n                  <\/jats:p>","DOI":"10.1007\/s00415-022-11431-1","type":"journal-article","created":{"date-parts":[[2022,11,4]],"date-time":"2022-11-04T05:02:46Z","timestamp":1667538166000},"page":"1004-1010","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Clinical and paraclinical features of small fiber neuropathy in Sj\u00f6gren\u2019s syndrome"],"prefix":"10.1007","volume":"270","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6018-6826","authenticated-orcid":false,"given":"Tabea","family":"Seeliger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrike Neelke","family":"Dreyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janna Margaretha","family":"Siemer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lena","family":"B\u00f6nig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Gingele","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maike Franziska","family":"Dohrn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nils","family":"Prenzler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Ernst","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"}]}],"member":"297","published-online":{"date-parts":[[2022,11,4]]},"reference":[{"key":"11431_CR1","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1136\/annrheumdis-2016-210571","volume":"76","author":"CH Shiboski","year":"2017","unstructured":"Shiboski CH, Shiboski SC, Seror R et al (2017) 2016 American College of Rheumatology\/European League Against Rheumatism classification criteria for primary Sj\u00f6gren\u2019s syndrome. 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Outside the submitted work, the authors received honoraria for lectures, travel grants, or research grants. TSe received a travel support to attend a scientific meeting by Abbvie, honoraria for preparation of a manuscript by Springer and was funded by the Ellen-Schmidt-Scholarship of the Hannover Medical School. JMS received a travel grant by the Gesellschaft der Freunde der MHH e.V. SG reports research support from Alnylam Pharmaceuticals, CSL Behring, Else Kr\u00f6ner Fresenius Foundation, Deutsche Forschungsgemeinschaft and Hannover Biomedical Research School (HBRS) and honoraria for lectures from Alnylam and Merck. MFD received financial reimbursement for consulting and advisory board activities and travel support to attend scientific meetings by Akcea Therapeuticals Inc., Alnylam Pharmaceuticals Inc., Amicus Therapeutics, and Pfizer Pharmaceuticals. MFD further received research funding by Pfizer Pharmaceuticals (ASPIRE 2018). DE received presentation honoraria from Abbvie, Amgen, BMS, Chugai, Cilag-Janssen, Galapagos, GSK, Medac, Lilly, Pfizer, Novartis, Roche and participated in advisory boards for Abbvie, Galapagos, Amgen and Novartis. TW received honoraria for lectures by Abbvie, BMS, Chugai, Galapagos, Janssen, Lilly, Pfizer, UCB and Roche. TSk reports research support from Bristol-Myers Squibb, Claudia von Schilling Foundation, Else Kr\u00f6ner Fresenius Foundation, and Sanofi Aventis, and honoraria for lectures and travel grants from Alexion, Alnylam, Bayer Vital, Biogen, Celgene, CSL Behring, Euroimmun, Janssen, Merck, Novartis, Pfizer, Roche, Sanofi Aventis, Siemens, and Sobi. HND, LB, NP and TW have nothing to declare.","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).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical statement"}}]}}