Pos1237 Peripheral Nervous System Involvement of Sjögren Disease: Characterization at the Time of Diagnosis in 16,703 Patients From the Sjögren Big Data Registry

Background: Neurological manifestations can occur in up to half of patients with Sjögren disease (SjD) and the peripheral nervous system (PNS) is the most commonly involved (5-21%). Objectives: To analyze the frequency and phenotypic expression of PNS involvement at the time of SjD diagnosis Methods: The Big Data Project Consortium is an international, multicenter 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. Results: By December 2023, the participant centers had included 16'703 patients from 28 countries (15'602 women, mean age at diagnosis of 51.66 years). PNS involvement at diagnosis, defined according to the ESSDAI classification, was reported in 869 (5.2%) patients. Among them, 518 (60%) showed mild active PNS involvement (pure sensory axonal polyneuropathy, V neuralgia, or proven small fibre neuropathy), 264 (30%) showed moderate involvement (axonal sensory–moto neuropathy, cryoglobulinemic pure sensory neuropathy, mild/moderate ganglionopathy, mild chronic inflammatory demyelinating polyneuropathy -CIDP-, or other cranial nerve involvements), and 87 (10%) showed highly active PNS involvement (severe motor axonal neuropathy, mononeuritis multiplex, severe ataxia due to ganglionopathy, or severe CIDP). Univariate analysis showed that patients with PNS involvement were more frequently men, diagnosed at an older age, white, had a higher frequency of activity in all the 12 ESSDAI domains and more commonly hypocomplementemia and cryoglobulinemia (all P-values Conclusion: Around 5% of patients had PNS involvement at the time of diagnosis of SjD. Patients with PNS involvement showed higher systemic activity in multiple domains (1.3-2 times higher frequency of constitutional, joint, renal, and muscular involvement), especially concerning concomitant CNS involvement (5-fold higher frequency). Hypocomplementemia was the key immunological marker independently associated with PNS involvement. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.