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Sjögren’s syndrome with and without neurological involvement
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 thos...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10188418/ https://www.ncbi.nlm.nih.gov/pubmed/36802030 http://dx.doi.org/10.1007/s00415-023-11613-5 |
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author | Seeliger, Tabea Kramer, Emelie Konen, Franz Felix Zehrfeld, Nadine Beider, Sonja Prenzler, Nils Kristian Gödecke, Vega Witte, Torsten Skripuletz, Thomas Ernst, Diana |
author_facet | Seeliger, Tabea Kramer, Emelie Konen, Franz Felix Zehrfeld, Nadine Beider, Sonja Prenzler, Nils Kristian Gödecke, Vega Witte, Torsten Skripuletz, Thomas Ernst, Diana |
author_sort | Seeliger, Tabea |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-10188418 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-101884182023-05-18 Sjögren’s syndrome with and without neurological involvement Seeliger, Tabea Kramer, Emelie Konen, Franz Felix Zehrfeld, Nadine Beider, Sonja Prenzler, Nils Kristian Gödecke, Vega Witte, Torsten Skripuletz, Thomas Ernst, Diana J Neurol Original Communication 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. Springer Berlin Heidelberg 2023-02-18 2023 /pmc/articles/PMC10188418/ /pubmed/36802030 http://dx.doi.org/10.1007/s00415-023-11613-5 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Original Communication Seeliger, Tabea Kramer, Emelie Konen, Franz Felix Zehrfeld, Nadine Beider, Sonja Prenzler, Nils Kristian Gödecke, Vega Witte, Torsten Skripuletz, Thomas Ernst, Diana Sjögren’s syndrome with and without neurological involvement |
title | Sjögren’s syndrome with and without neurological involvement |
title_full | Sjögren’s syndrome with and without neurological involvement |
title_fullStr | Sjögren’s syndrome with and without neurological involvement |
title_full_unstemmed | Sjögren’s syndrome with and without neurological involvement |
title_short | Sjögren’s syndrome with and without neurological involvement |
title_sort | sjögren’s syndrome with and without neurological involvement |
topic | Original Communication |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10188418/ https://www.ncbi.nlm.nih.gov/pubmed/36802030 http://dx.doi.org/10.1007/s00415-023-11613-5 |
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