BACKGROUND: Patients with immune-mediated inflammatory diseases such as rheumatoid arthritis or systemic lupus are at increased risk of cardiovascular disease. However, the cardiovascular risk of patients with primary Sjögren syndrome (pSS) remains poorly studied. We aimed to investigate the association between pSS and cardiovascular morbidity and mortality. METHODS: We performed a systematic review of articles in MEDLINE and the COCHRANE library and recent abstracts from US and European meetings, searching for reports of randomized controlled studies of cardiovascular morbidity and cardiovascular mortality in pSS. The relative risk (RR) values for cardiovascular morbidity and mortality associated with pSS were collected and pooled in a meta-analysis with a random-effects model by using Review Manager (Cochrane collaboration). RESULTS: The literature search revealed 484 articles and abstracts of interest; 14 studies (67 124 pSS patients) were included in the meta-analysis. With pSS versus control populations, the risk was significantly increased for coronary morbidity (RR= 1.34, 95%CI: 1.06-1.38; p = 0.01), cerebrovascular morbidity (RR= 1.46, 95%CI: 1.43-1.49, p<0.00001), heart failure rate (odds ratio=2.54, 95%CI: 1.30-4.97, p<0.007), and thromboembolic morbidity (RR= 1.78, 95%CI: 1.41-2.25, p<0.00001), with no statistically significant increased risk of cardiovascular mortality (RR= 1.48, 95%CI: 0.77-2.85, p=0.24). CONCLUSION: This meta-analysis demonstrates that pSS is associated with increased cardiovascular morbidity, which suggests that these patients should be screened for cardiovascular comorbidities and considered for preventive interventions, in a multidisciplinary approach with cardiologists. This article is protected by copyright. All rights reserved.