Ab0808 Central Nervous System Involvement in Primary Sjögren’s Syndrome: A Single Center Experience From Turkey

Background: Primary Sjögren's Syndrome (pSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and a variable spectrum of extraglandular manifestations (1, 2). Focal/multifocal, diffuse and multiple sclerosis (MS) like diseases are included in the central nervous system (CNS) involvement of pSS spectrum. The frequency of CNS involvement has been reported 2.8% to 48 % in different studies (3-5). Objectives: Studies on characteristics and treatment options for CNS involvement in pSS are limited on a case-by-case basis. Our aim is to present the characteristics and prognosis of patients with central nervous system involvement within our Sjögren's syndrome cohort. Methods: The patients who followed up with pSS in the Department of Rheumatology at the Ankara University Faculty of Medicine, between January 2010 and December 2023 were retrospectively screened. A total of 427 patients were classified according to the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Primary Sjögren Classification Criteria(6). Patients who demonstrated CNS involvement at any time during diagnosis and follow-up were selected. After excluding causes secondary to CNS involvement, the clinical decisions were made in consultation with neurology and psychiatry. Demographic, clinical, seroimmunological characteristics; neuroimaging and electromyogram/nerve conduction study data and cerebrospinal fluid (CSF) analysis of the patients were collected and analyzed. Results: CNS involvement was diagnosed in 15 out of 427 patients (3.5%). The median age of CNS involvement was 50 years and 13 (86.7%) of them were female. Patient characteristics are in Table 1. Patients' neurologic patern, neuroimaging data, treatment and outcome are in Table 2. Neurological symptoms were the initial manifestation in 80% of the patients. Ro-52 was positive in 9 patients (60%) and was the most frequent autoantibody. MS-like pattern and diffuse pattern were most common patterns, both were seen in %40. Any immunosuppressive therapy was given to 80% of the patients, and 20% of them received symptomatic treatment. Only 6.7% of patients worsened despite immunosuppressive treatment, 26.7% were stable, and 53.3% clinically improved. Conclusion: CNS involvement in pSS is rare and reported in small numbers because it can be seen in different patterns and resembles variable neurological clinical pictures. In order to better determine treatment response, options, and prognosis, studies with a larger patient group and collaboration between neurology and rheumatology for patient follow-up are needed. REFERENCES: 1 Patel R, Shahane A. The epidemiology of Sjögren's syndrome. Clin Epidemiol. 2014;6:247-55. 2 Fox RI. Sjögren's syndrome. Lancet. 2005;366(9482):321-31. 3 Govoni M, Padovan M, Rizzo N, Trotta F. CNS involvement in primary Sjögren's syndrome: prevalence, clinical aspects, diagnostic assessment and therapeutic approach. CNS Drugs. 2001;15(8):597-607. 4 Massara A, Bonazza S, Castellino G, Caniatti L, Trotta F, Borrelli M, et al. 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). 2010;49(8):1540-9. 5 Yayla ME, Karaman Z, Torgutalp M, Keleşoğlu Dinçer AB, Aydemir Gülöksüz EG, Sezer S, et al. Early onset primary Sjögren syndrome, clinical and laboratory characteristics. Clin Rheumatol. 2020;39(9):2689-96. 6 Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al. 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. 2017;69(1):35-45. Table 1. Demographic and clinical results of patients pSS with CNS involvement CNS: Central nervous system, PNS: Peripheral nervous system Table 2. Patients neurologic patern, neuroimaging data, treatment and outcome Acknowledgements: NIL. Disclosure of Interests: None declared.