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. 2023 Jun;270(6):2987-2996.
doi: 10.1007/s00415-023-11613-5. Epub 2023 Feb 18.

Sjögren's syndrome with and without neurological involvement

Tabea Seeliger #  1 , Emelie Kramer #  2 , Franz Felix Konen  1 , Nadine Zehrfeld  2 , Sonja Beider  2 , Nils Kristian Prenzler  3 , Vega Gödecke  4 , Torsten Witte  2 , Thomas Skripuletz #  5 , Diana Ernst #  2
Affiliations

Sjögren's syndrome with and without neurological involvement

Tabea Seeliger et al. J Neurol. 2023 Jun.

Abstract

Objective: Neurological manifestations of Sjögren's syndrome can be severe but also treatment-responsive. We aimed to systematically evaluate neurological manifestations of primary Sjögren's syndrome and find clinical features allowing sufficient identification of affected patients (pSSN) among those with Sjögren's syndrome without neurological involvement (pSS).

Methods: Para-/clinical features of patients with primary Sjögren's 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 Sjögren's syndrome, and newly diagnosed pSS patients are thoroughly evaluated for neurologic involvement. pSSN disease activity was rated by the Neurological Involvement of Sjögren's Syndrome Disease Activity Score (NISSDAI).

Results: 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 Sjögren's syndrome were male sex [p < 0.001], older age at disease onset [p < 0.0001], hospitalization at first presentation [p < 0.001], lower IgG levels [p = 0.04] and higher eosinophil values (treatment-naïve) [p = 0.02]. Univariate regression additionally showed older age at diagnosis [p < 0.001], lower prevalence of rheumatoid factor [p = 0.001], SSA(Ro)/SSB(La) antibodies [p = 0.03; p < 0.001], higher white blood cell count [p = 0.02] and CK levels [p = 0.02] (treatment-naïve) in pSSN.

Interpretation: 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 Sjögren's syndrome has been underestimated. Intensified screening for neurologic involvement should be included in the diagnostic algorithm for Sjögren's syndrome, especially in males of older age and with severe disease course requiring hospitalization.

Keywords: Neuro-Sjögren; Neurological manifestations of Sjögren’s syndrome; Primary Sjögren’s syndrome; Sjögren-associated neuropathy.

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Conflict of interest statement

The authors declare no relevant conflicts of interest. 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 Kröner Fresenius Foundation. NZ was funded by the Else Kröner Fresenius Foundation. TSk received research grants by Alnylam Pharmaceuticals, Bristol-Myers Squibb Foundation for Immuno-Oncology, Claudia von Schilling Foundation, CSL Behring, Else Kröner Fresenius Foundation, Novartis, Sanofi Aventis, VHV Stiftung. SB has nothing to disclose.

Figures

Fig. 1
Fig. 1
Frequency of neurological features in all 512 included patients, with blue rectangle indicating features that led to the allocation to the subgroup of Sjögren’s syndrome with neurological involvement. pSS primary Sjögren’s syndrome, pSSN primary Sjögren’s syndrome with neurological involvement, NCS nerve conduction studies, TIA Transient Ischemic Attack, NMOSD Neuromyelitis Optica Spectrum Disease
See this image and copyright information in PMC

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