AB0823 PREMATURE CEREBROVASCULAR EVENTS IN PRIMARY SJÖGREN SYNDROM (pSS) ARE ASSOCIATED WITH DELAYED DIAGNOSIS OF pSS
Authors: S. Beider, C. L. Zippel, N. Zehrfeld, F. Tapken, T. Seeliger, T. Skripuletz, T. Witte, D. Ernst
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Primary Sjögren´s syndrome (pSS) is associated with an increased risk of cerebrovascular events (CVE) (Bartoloni et al., 2015; Yong et al., 2018). Existing data from our center suggested an association between pSS and premature stroke, with a median age of 55.2 (Zippel et al., 2022). The heterogeneous nature of the signs and symptoms of pSS often causes delay in diagnosis, which remains a serious issue for pSS patients and results in prolongation of patients' distress (Komori et al., 2021). <h3>Objectives:</h3> To evaluate differences in patients above and below the age of 50 with cerebrovascular events in pSS. <h3>Methods:</h3> Between August 2018 and September 2023, 548 consecutive patients fulfilling ACR/EULAR classification criteria for pSS were enrolled in the study. Data were collected using standardized institutional protocols and review of medical records. Two groups were compared: patients with stroke &lt; 50 (young stroke) and patients who experienced a CVE ≥ 50 years (old stroke). Statistical analysis was performed using SPSS 29 (IBM, Armonk, NY, USA). <h3>Results:</h3> Of 548 confirmed pSS patients, 37 (6.8%) with a history of CVE, including ischemic stroke were identified. The patients median age was 60 years while the median age at the occurrence of stroke was 53. Stroke &lt;40 years of age occurred in 7/37 (18.9%) patients, between 40-50 years of age accounted for a further 21.6%. Patients with young stroke (15/37, 40.5 %) were in average 25 years younger than patients with old stroke (22/37, 59.5%) with median age 41 vs. 66.5 years (p&lt;0.001). Remarkably, 18/37 (48.6%), i.e.10/15 (66.7%) vs. 8/22 (36.4%), p=0.070) of pSS stroke patients experienced stroke before the diagnosis of pSS, even though all patients suffered sicca symptoms at time of CVE. The time interval between the initial onset of pSS symptoms and the confirmation of pSS diagnosis was notably prolonged in the younger group, with a median duration of four years. In contrast, in patients that initially presented with pSS and later CVE, the time between initial pSS manifestation and the diagnosis was significantly shorter, less than one year (p=0.017). Patients experiencing strokes before the diagnosis of pSS were significantly younger (18/37, 49.6 years vs. 19/37, 64 years, p=0.037). Interestingly both groups showed no significant differences in cardiovascular risks or disease activity (Table 1). The ESSPRI and ESSDAI scores were similar in both groups. Young stroke tended to exhibit a higher prevalence of vasculitis compared to old stroke (3/15, 20 % vs. 2/22, 9.1%), as well as an increased occurrence of thrombosis (4/15, 26.7% vs. 2/22, 9.1%). The small cohort size warrants careful interpretation of the results. <h3>Conclusion:</h3> pSS patients with CVE below the age 50 often received their pSS diagnosis delayed, even after the CVE, even though the initial manifestation of pSS occurred in all patients before the stroke. Our results suggest that screening for pSS in patients with stroke may be useful. <b>Table 1.</b> <h3>REFERENCES:</h3> [1] Bartoloni, E., Baldini, C., Schillaci, G., et al. (2015). Cardiovascular disease risk burden in primary Sjögren's syndrome: results of a population-based multicentre cohort study. J Intern Med, 278(2), 185-192. doi:10.1111/joim.12346 [2] Komori, K., Komori, M., Horino, T., et al. (2021). Factors associated with delayed diagnosis of Sjögren's syndrome among members of the Japanese Sjögren's Association for Patients. Clin Exp Rheumatol, 39 Suppl 133(6), 146-152. doi:10.55563/clinexprheumatol/s8l2n0 [3] Yong, W. C., Sanguankeo, A., &amp; Upala, S. (2018). Association between primary Sjögren's syndrome, cardiovascular and cerebrovascular disease: a systematic review and meta-analysis. Clin Exp Rheumatol, 36 Suppl 112(3), 190-197. [4] Zippel, C. L., Beider, S., Kramer, E., et al. (2022). Premature stroke and cardiovascular risk in primary Sjögren's syndrome. Front Cardiovasc Med, 9, 1048684. doi:10.3389/fcvm.2022.1048684 <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>