POS0852 NEUROLOGICAL INVOLVEMENT IN SJÖGREN'S DISEASE: PREVALENCE AND KEY CLINICAL ASSOCIATIONS FROM A SINGLE-CENTER COHORT
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Background: Sjögren's Disease (SD) is a chronic autoimmune condition primarily targeting the exocrine glands, most notably the lacrimal and salivary glands. Beyond its characteristic presentation of ocular and oral dryness, SD can involve both peripheral (PNS) and central nervous systems (CNS). Peripheral neuropathies frequently observed in SD include sensory ataxic neuropathy (SAN), painful sensory neuropathy (PSN), multiple mononeuropathy (MMN), and autonomic neuropathy (AN). Central manifestations span an array of symptoms, from mild cognitive disturbances or MS-like presentations to severe encephalitis. These varied presentations suggest a complex, multifactorial pathogenesis that may significantly affect patient outcomes. Objectives: To estimate the prevalence of neurological involvement among SD patients and identify clinical, immunological, and disease activity factors associated with these manifestations, with a focus on "NeuroSjögren" (NS)—neurological compromise definitively attributed to SD. Methods: A single-center retrospective review was conducted on 199 patients fulfilling SD classification criteria. Demographic, comorbidity, and immunologic data were extracted from medical records. Neurological findings included peripheral neuropathy (PNP), myopathy (MYO), sensory-neural hearing loss (SNHL), myasthenia gravis (MG), and CNS involvement. NS was defined as neurological involvement directly attributable to SD after excluding alternative etiologies (e.g., infections, vitamin deficiencies, inherited disorders). Associations were evaluated with chi-square or z-score tests (p<0.05). Results: Of the 199 patients who fulfilled the classification criteria for SD, the majority were female (90.95%) with a mean age of 56.19 years. Immunological evaluations revealed high rates of antinuclear antibodies (79.40%), anti-SSA/Ro (59.30%), anti-SSB/La (18.56%), rheumatoid factor (31.29%), hypergammaglobulinemia (25.63%) and hypocomplementemia (24.12%). Histologically, 74.47% presented focal lymphocytic sialadenitis, with a mean focus score of 1.87 (±1.63). Overall neurological involvement was documented in 30.15% of patients. Among these, 12.05% qualified as NS. PNP and MYO each occurred in 7.04%, SNHL in 6.53%, and both MG and CNS manifestations in 2.51%. PNP emerged as the most frequent PNS abnormality, encompassing SAN, PSN, and AN phenotypes. Renal involvement (p=0.015) and nephritis (p=0.004) were significantly linked to neurological disease. NS was associated with older age (p=0.048), male sex (p=0.032), cryoglobulinemia (p=0.010), antiphospholipid antibodies (p=0.002), renal involvement (p=0.045), and higher ESSDAI scores (p=0.023). Additional analyses revealed that cryoglobulinemia also correlated with PNP (p<0.001). MG showed significant association with mortality (p<0.001) and SNHL (p=0.002). Furthermore, antiphospholipid antibodies were linked to CNS involvement (p<0.001) and spinal cord compromise (p<0.001). In contrast, markers such as antinuclear antibodies, SSA/Ro, SSB/La and rheumatoid factor did not demonstrate a significant relationship with NS. Conclusion: Neurological manifestations affected nearly one-third of these SD patients, with NS representing 12% of the total cohort. The significant correlations of NS with cryoglobulinemia, antiphospholipid antibodies, renal disease, and higher ESSDAI suggest a more severe clinical phenotype. These findings underscore the importance of vigilant neurologic screening and prompt multidisciplinary management to optimize outcomes in SD. REFERENCES: [1] Margaretten M. Neurologic Manifestations of Primary Sjögren Syndrome. Rheum Dis Clin North Am. 2017;43(4):519-529. doi:10.1016/j.rdc.2017.06.002. [2] Morreale M, Marchione P, Giacomini P, et al. Neurological involvement in primary Sjögren syndrome: a focus on central nervous system. PLoS One. 2014;9(1):e84605. Published 2014 Jan 20. doi:10.1371/journal.pone.0084605. [3] Gono T, Kawaguchi Y, Katsumata Y, et al. Clinical manifestations of neurological involvement in primary Sjögren's syndrome. Clin Rheumatol. 2011;30(4):485-490. doi:10.1007/s10067-010-1458-7. Acknowledgements: NIL . Disclosure of Interests: None declared . © The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.
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DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- POS0852 NEUROLOGICAL INVOLVEMENT IN SJÖGREN'S DISEASE: PREVALENCE AND KEY CLINICAL ASSOCIATIONS FROM A SINGLE-CENTER COHORT
- Date Crossref
- 01/06/2025
- Éditeur
- Elsevier BV
- Type
- journal-article
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