Background/Purpose: Primary Sjögren’s syndrome (pSS) is an autoimmune disorder characterized by chronic multisystem inflammation with shared pathophysiology with SLE and RA. Cardiovascular events have emerged as major causes of morbidity and mortality in patients with autoimmune diseases however, the clinical significance of cardiovascular disease in patients with pSS remains unclear. We aim to study the association between cardiovascular disease and primary Sjögren’s syndrome (pSS) and analyze the risk of cardiovascular disease accordingly to glandular/extraglandular involvement and anti-Ro/SSA and/or Anti-La/SSB autoantibody status.

Methods: pSS patients fulfilling the 2016 ACR/EULAR classification criteria for pSS were consecutively evaluated and followed in our department between 2000 and 2022. We evaluated the prevalence and clinical significance of cardiovascular risk factors with primary Sjögren’s syndrome (SS), focusing on the possible association with clinical and immunological features, the therapies administered, and the impact on cardiovascular disease. A two-tailed value of p< 0.05 was taken to indicate statistical significance. Potential risk factors associated with cardiovascular involvement were determined by multivariate regression analyses.

Results: A total of 102 pSS patients were included. 90% were female, with a mean age of 65±24 years and a disease duration of 9.9 ±7 years. Patients with extraglandular involvement had a higher prevalence of cardiovascular risk factors, including arterial hypertension (OR 2.28 95% CI (1.01-5.09), p 0.04), dyslipidemia (OR 4.4 95% CI (1.67-11.6), p 0.003), higher LDL mean values (116±48 vs 99±44, p 0.038), uric acid (6.58±1.7 vs 4.3±1.03, p 0.04) and higher risk for myocardial ischemia (OR 4.09 95% CI (1.46-11.4), p 0.01) after adjustment for age, sex, disease duration, and the significant variables in the univariate analysis. Patients positive for both Ro/SSA and La/SSB autoantibodies had a substantially higher risk of arrythmia (OR 3.4 95% CI (1.01-10.6), p 0.04), arterial and venous thromboembolism (OR 5.5 95% (1.18-25.7), p 0.03) and stroke (OR 3 95% (1.02-8.8), p 0.04). In the multivariate logistic regression analysis, extraglandular organ involvement (p=0.008), beta2microglobulin levels (p=0.001), hypocomplementemia of C3 (p=0.01), the use of glucorticoids (p=0.02), hypergammaglobulinemia (p=0.02), ESR levels (p=0.007), treatment with HCQ (p=0.03) and an ESSDAI (Sjögren’s syndrome disease activity index) >13 (p=0.02) were found to be factors associated with increased or decreased odds ratio forcardiovascular events in pSS patients. Anti-Ro/SSA and anti-La/SSB were significant predictors in univariate but not in multivariate analysis.

Conclusion: pSS patients are more vulnerable to cardiovascular diseases (CVDs). In addition to traditional CVD risk factors, we identified risk factors independently associated with cardiovascular involvement in pSS patients, which suggests the need for early detection and prevention measures to improve the prognosis in those patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/extraglandular-involvement-and-autoantibody-status-as-risk-factors-for-cardiovascular-disease-in-primary-sjogrens-syndrome-pss-a-20-year-follow-up-study/