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Open Access 01-02-2025 | Primary Sjögren Syndrome | Case Based Review

Diversity of central nervous system manifestations in Sjogren’s Disease: a case-based review

Authors: Marta Jaskólska, Magdalena Rytlewska, Natalia Aleksandra Dułak, Miłosz Ulanowski, Mariusz Kwarciany, Mariusz Stanisław Wiglusz, Wiesław Jerzy Cubała, Michał Chmielewski

Published in: Rheumatology International | Issue 2/2025

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Abstract

Sjogren’s disease (SjD) is a chronic and disabling autoimmune disease, predominantly characterized by dryness of the mouth and eyes, resulting from lymphocytic infiltration of exocrine glands. While these are the most prominent symptoms, extra-glandular manifestations are also common. Studies suggest that up to 70% of SjD patients experience neurological symptoms, which interestingly often precede the hallmark dryness. Although every structure of the nervous system can be affected, disorders of peripheral nervous system (PNS) are more common than central nervous system (CNS) involvement. The CNS manifestations can range from subtle to severe, with some patients experiencing a rapid progression of symptoms. The literature documents cases where patients initially diagnosed with Creutzfeldt-Jakob disease, neurosarcoidosis, temporary hemiplegia, neuromyelitis optica, or epilepsy were ultimately diagnosed as having SjD. Here, we present five SjD patient cases, each with a different manifestation of CNS involvement, along with an overview of the current understanding of CNS disorders in the course of the disease. In four cases, the neurological manifestations appeared before the onset of sicca symptoms. In one patient, diagnosis was delayed by 15 years due to an atypical presentation. After receiving immunosuppression, all patients experienced notable alleviation of their symptoms.
Literature
4.
go back to reference Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E, Gottenberg JE, Bootsma H, Mariette X, Vitali C (2010); EULAR Sjögren’s Task Force. EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren’s syndrome. Ann Rheum Dis. 69(6):1103-9. https://doi.org/10.1136/ard.2009.110619 Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E, Gottenberg JE, Bootsma H, Mariette X, Vitali C (2010); EULAR Sjögren’s Task Force. EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren’s syndrome. Ann Rheum Dis. 69(6):1103-9. https://​doi.​org/​10.​1136/​ard.​2009.​110619
5.
go back to reference Ramos-Casals M, Brito-Zerón P, Solans R, Camps MT, Casanovas A, Sopeña B, Díaz-López B, Rascón FJ, Qanneta R, Fraile G, Pérez-Alvarez R, Callejas JL, Ripoll M, Pinilla B, Akasbi M, Fonseca E, Canora J, Nadal ME, de la Red G, Fernández-Regal I, Jiménez-Heredia I, Bosch JA, Ayala MD, Morera-Morales L, Maure B, Mera A, Ramentol M, Retamozo S, Kostov B; SS Study Group (2014); Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI). Systemic involvement in primary Sjogren’s syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford). 53(2):321 − 31. https://doi.org/10.1093/rheumatology/ket349 Ramos-Casals M, Brito-Zerón P, Solans R, Camps MT, Casanovas A, Sopeña B, Díaz-López B, Rascón FJ, Qanneta R, Fraile G, Pérez-Alvarez R, Callejas JL, Ripoll M, Pinilla B, Akasbi M, Fonseca E, Canora J, Nadal ME, de la Red G, Fernández-Regal I, Jiménez-Heredia I, Bosch JA, Ayala MD, Morera-Morales L, Maure B, Mera A, Ramentol M, Retamozo S, Kostov B; SS Study Group (2014); Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI). Systemic involvement in primary Sjogren’s syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford). 53(2):321 − 31. https://​doi.​org/​10.​1093/​rheumatology/​ket349
11.
go back to reference Carvajal Alegria G, Guellec D, Mariette X, Gottenberg JE, Dernis E, Dubost JJ, Trouvin AP, Hachulla E, Larroche C, Le Guern V, Cornec D, Devauchelle-Pensec V, Saraux A (2016). Epidemiology of neurological manifestations in Sjögren’s syndrome: data from the French ASSESS Cohort. RMD Open. 2(1):e000179. https://doi.org/10.1136/rmdopen-2015-000179 Carvajal Alegria G, Guellec D, Mariette X, Gottenberg JE, Dernis E, Dubost JJ, Trouvin AP, Hachulla E, Larroche C, Le Guern V, Cornec D, Devauchelle-Pensec V, Saraux A (2016). Epidemiology of neurological manifestations in Sjögren’s syndrome: data from the French ASSESS Cohort. RMD Open. 2(1):e000179. https://​doi.​org/​10.​1136/​rmdopen-2015-000179
18.
go back to reference Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X (2017); International Sjögren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 69(1):35–45. https://doi.org/10.1002/art.39859 Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X (2017); International Sjögren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 69(1):35–45. https://​doi.​org/​10.​1002/​art.​39859
29.
34.
go back to reference Massara A, Bonazza S, Castellino G, Caniatti L, Trotta F, Borrelli M, Feggi L, Govoni M (2010). Central nervous system involvement in Sjögren’s syndrome: unusual, but not unremarkable–clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology (Oxford). 49(8):1540-9. https://doi.org/10.1093/rheumatology/keq111 Massara A, Bonazza S, Castellino G, Caniatti L, Trotta F, Borrelli M, Feggi L, Govoni M (2010). Central nervous system involvement in Sjögren’s syndrome: unusual, but not unremarkable–clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology (Oxford). 49(8):1540-9. https://​doi.​org/​10.​1093/​rheumatology/​keq111
38.
go back to reference Kchaou M, Ben Ali N, Hmida I, Fray S, Jamoussi H, Jalleli M, Echebbi S, Achouri A, Belal S (2015). Parkinsonism and Sjögren’s Syndrome: A Fortuitous Association or a Shared Immunopathogenesis? Case Rep Med. 2015:432910. https://doi.org/10.1155/2015/432910 Kchaou M, Ben Ali N, Hmida I, Fray S, Jamoussi H, Jalleli M, Echebbi S, Achouri A, Belal S (2015). Parkinsonism and Sjögren’s Syndrome: A Fortuitous Association or a Shared Immunopathogenesis? Case Rep Med. 2015:432910. https://​doi.​org/​10.​1155/​2015/​432910
41.
go back to reference Tzarouchi LC, Tsifetaki N, Konitsiotis S, Zikou A, Astrakas L, Drosos A, Argyropoulou MI (2011). CNS involvement in primary Sjogren Syndrome: assessment of gray and white matter changes with MRI and voxel-based morphometry. AJR Am J Roentgenol. 197(5):1207-12. https://doi.org/10.2214/AJR.10.5984 Tzarouchi LC, Tsifetaki N, Konitsiotis S, Zikou A, Astrakas L, Drosos A, Argyropoulou MI (2011). CNS involvement in primary Sjogren Syndrome: assessment of gray and white matter changes with MRI and voxel-based morphometry. AJR Am J Roentgenol. 197(5):1207-12. https://​doi.​org/​10.​2214/​AJR.​10.​5984
42.
go back to reference Alhomoud IA, Bohlega SA, Alkawi MZ, Alsemari AM, Omer SM, Alsenani FM (2009). Primary Sjogren’s syndrome with central nervous system involvement. Saudi Med J. 30(8):1067-72. PMID: 19668890. Alhomoud IA, Bohlega SA, Alkawi MZ, Alsemari AM, Omer SM, Alsenani FM (2009). Primary Sjogren’s syndrome with central nervous system involvement. Saudi Med J. 30(8):1067-72. PMID: 19668890.
43.
46.
go back to reference Pars K, Pul R, Schwenkenbecher P, Sühs KW, Wurster U, Witte T, Bronzlik P, Stangel M, Skripuletz T (2017). Cerebrospinal Fluid Findings in Neurological Diseases Associated with Sjögren’s Syndrome. Eur Neurol. 77(1–2):91–102. https://doi.org/10.1159/000454765 Pars K, Pul R, Schwenkenbecher P, Sühs KW, Wurster U, Witte T, Bronzlik P, Stangel M, Skripuletz T (2017). Cerebrospinal Fluid Findings in Neurological Diseases Associated with Sjögren’s Syndrome. Eur Neurol. 77(1–2):91–102. https://​doi.​org/​10.​1159/​000454765
47.
go back to reference Tjensvoll AB, Lauvsnes MB, Zetterberg H, Kvaløy JT, Kvivik I, Maroni SS, Greve OJ, Beyer MK, Hirohata S, Putterman C, Alves G, Harboe E, Blennow K, Gøransson LG, Omdal R (2021). Neurofilament light is a biomarker of brain involvement in lupus and primary Sjögren’s syndrome. J Neurol. 268(4):1385–1394. https://doi.org/10.1007/s00415-020-10290-y Tjensvoll AB, Lauvsnes MB, Zetterberg H, Kvaløy JT, Kvivik I, Maroni SS, Greve OJ, Beyer MK, Hirohata S, Putterman C, Alves G, Harboe E, Blennow K, Gøransson LG, Omdal R (2021). Neurofilament light is a biomarker of brain involvement in lupus and primary Sjögren’s syndrome. J Neurol. 268(4):1385–1394. https://​doi.​org/​10.​1007/​s00415-020-10290-y
48.
50.
Metadata
Title
Diversity of central nervous system manifestations in Sjogren’s Disease: a case-based review
Authors
Marta Jaskólska
Magdalena Rytlewska
Natalia Aleksandra Dułak
Miłosz Ulanowski
Mariusz Kwarciany
Mariusz Stanisław Wiglusz
Wiesław Jerzy Cubała
Michał Chmielewski
Publication date
01-02-2025
Publisher
Springer Berlin Heidelberg
Published in
Rheumatology International / Issue 2/2025
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-024-05753-8

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