POS1247 CHARACTERIZATION OF 284 PATIENTS PRESENTING WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT AT DIAGNOSIS OF SJÖGREN DISEASE: RESULTS FROM THE SJÖGREN BIG DATA REGISTRY

Background: Central nervous system (CNS) involvement is one of the numerous extra-glandular manifestations of primary Sjögren disease (SjD) and it has been described in 8-70% of patients at various stages of the disease. Objectives: To characterize how CNS involvement presents at the diagnosis of SjD. Methods: The Big Data Sjögren Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centers on clinical research in SjD of the five continents was collected as a first step. The centers share a harmonized data architecture and conduct cooperative online efforts to refine collected data under the coordination of a big data statistical team. The inclusion criteria were the fulfillment of the 2002 or 2016 classification criteria. CNS involvement at diagnosis was defined according to the EULAR Sjögren syndrome disease activity index (ESSDAI) classification. Results: By December 2023, 16,703 patients from 28 countries (15'602 women, mean age at diagnosis of 51.66 years) were included. Among them, 284 (1.7%) showed CNS involvement at the rime of SjD diagnosis, including 173 with moderate involvement (cranial nerve involvement of central origin, optic neuritis, or multiple sclerosis-like syndrome with symptoms restricted to pure sensory impairment or proven cognitive impairment) and 111 with highly active CNS involvement (symptomatic cerebral vasculitis, seizures, transverse myelitis, lymphocytic meningitis, or multiple sclerosis-like syndrome with motor deficit). Univariate analysis showed that patients with CNS involvement were more frequently men and white, had the constitutional, renal, muscular, and peripheral nervous system ESSDAI domains more frequently involved, and more often low C3 values (all P-values <0.001). The multivariate logistic regression model adjusted for the epidemiological variables (age, sex, ethnicity) and the above-mentioned ESSDAI variables with p values <0.001 showed that activity in the renal (OR = 1.88; 95% CI, 1.07–3.30) and PNS (OR = 5.47; 95% CI, 3.75–7.99) ESSDAI domains were independently associated with a concomitant diagnosis of CNS involvement at the time of diagnosis of SjD. Conclusion: In a large sample of worldwide SjD patients, CNS involvement was present at disease diagnosis in less than 2% of patients, being one of the less frequent systemic involvements of the disease. Patients with CNS involvement at SjD diagnosis showed a 2-fold higher frequency of concomitant renal involvement and a 5-fold higher frequency of having concomitant PNS involvement. No serological marker was identified with the risk of being diagnosed with SjD-related CNS involvement. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.