Abstract: Abstract: Objective To investigate the clinical characteristics of nervous system involvement in patients with primary Sjogren’s syndrome (pSS). Methods Data of 32 pSS patients with nervous system involvement collected from Tianjin Medical University General Hospital and Peking Union Medical College Hospital were retrospectively analyzed. The demographic characteristics, clinical symptoms, laboratory parameters and imaging results were evaluated and analyzed. Results In 32 patients with pSS, there were 30 (93.8%) females and 2 (6.2%) males, with the average age 46±12 years. The neurological impairment was the first symptom in 16 (50%) patients, and sicca symptoms was the first symptom in the rest patients. Thirteen (40.6%) patients developed isolated peripheral nervous system (PNS) damages, 10 (31.3%) patients developed isolated central nervous system (CNS) damages, and 9 (40.6%) patients showed both (CNS-PNS). The most common lesion of PNS was polyneuropathy (6, 18.8%). The most common lesion of CNS was optic neuromyelitis pedigree disease (NMODS, 6, 18.8%). Patients with PNS lesion were more likely to have Reynolds phenomenon than those with CNS lesion (P=0.046). The prevalence of interstitial lung disease (ILD) was significantly higher in pSS patients with PNS and CNS-PNS damages than patients with CNS lesion (P=0.013). Conclusion Polyneuropathy and NMODS are the most common damages in pSS patients with PNS and CNS lesions respectively. Patients with PNS lesions are more likely to have ILD. ’

Key words: Sjogren’s syndrome, neurologic manifestations, central nervous system diseases, the clinical characteristics