CLINICAL FEATURES OF SJOGREN'S SYNDROME WITH AND WITHOUT NEUROLOGICAL INVOLVEMENT (NEURO-SJOGREN)

BackgroundSjögren’s Syndrome is well known for its characteristic sicca symptoms due to autoinflammatory destruction of the salivary and lacrymal glands, but neurological involvement is also common in this entity. Nevertheless, previously published smaller studies suggested distinct clinical features for Sjögren’s syndrome with and without neurological involvement, such as a more balanced gender distribution and lower IgG levels in patients with Sjögren‘ syndrome and neurological involvement1,2.ObjectivesWe therefore aimed to systematically assess clinical features of patients with Sjögren’s syndrome with and without neurological involvement, find relevant in-between group differences and hereby aid early detection of both patient groups in the clinical routine to facilitate further studies, potentially with new therapeutic approaches.MethodsWe retrospectively assessed patients with Sjögren’s syndrome treated at the neurological and rheumatological/ immunological departement of our university hospital between 05/2014 and 09/2021 for available laboratory and clinical data. The displayed data represent preliminary results of this ongoing study.Results405 patients, who fulfilled the current ACR/EULAR classification criteria for Sjögren’s syndrome3 were currently included in the study (median age 59years [IQR 50-70 years], median ESSDAI 10 [IQR 3-16]). 228 patients (56%) showed neurological involvement. They were significantly more often male (32% vs. 14%; p<0.001) and showed lower IgG serum levels (median 11 g/l [IQR 9-13 g/l] vs 12 g/l [IQR 10-16 g/l], p<0.01) in comparison to patients with Sjögren’s syndrome but without neurological involvement. However, presence of objective xerostomia, objective xerophthalmia, SSA(Ro)-antibody-positivity or sialadenits grade 3 or 4 (Chisholm and Mason) on salivary gland biopsy did not differ between the two groups.ConclusionPreliminary analysis of this ongoing study supports the hypothesis, that patients with Sjögren’s syndrome and neurological impairment might express a distinct clinical phenotype in comparison to patients with Sjögren’s syndrome but without neurological involvement.References[1]Sjögren’s syndrome should be considered in patients with motor neuropathy. Zeitschrift fur Rheumatologie 2020; 79: 707–709.[2]Seeliger T, Prenzler NK, Gingele S, et al. Neuro-Sjögren: Peripheral neuropathy with limb weakness in Sjögren’s syndrome. Front Immunol 2019; 10: 1600.[3]Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome. Ann Rheum Dis 2017; 76: 9–16.Disclosure of InterestsTabea Seeliger: None declared, Emelie Kramer: None declared, Franz F. Konen: None declared, Sonja Beider: None declared, Alexandra Jablonka: None declared, Torsten Witte: None declared, Thomas Skripuletz Employee of: Honoraria for lectures: Alexion, Alnylam, Bayer Vital, Biogen, Celgene, CSL Behring, Euroimmun, Janssen, Merck Serono, Novartis, Pfizer, Roche, Sanofi, Siemens, Diana Ernst Consultant of: Participation in Advisory Boards: Abbvie, Galapagos, Amgen, Novartis, Employee of: Fees for Presentations: Abbvie, Amgen, BMS, Chugai, Cilag-Janssen, Galapagos, GSK, Medac, Lilly, Pfizer, Novartis, Roche