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Objective: Behçet disease (BD) is a chronic inflammatory multiorgan disease. An increased risk of cardiovascular disease (CVD) and heightened death rate with BD have been suggested, but to our knowledge, a nationwide large-scale study has not been conducted to date. This study aimed to determine the overall CV risk and death rate in patients with BD versus controls using the Korean National Health Insurance Service claim database., Methods: Patients with BD (n = 5576) with no previous history of CVD were selected from 2010 to 2014. An age- and sex-matched control population of individuals without BD (n = 27,880) was randomly sampled at a ratio of 5:1. Both cohorts were followed for incident CVD or all-cause death until 2015., Results: The risks of myocardial infarction (HR 1.72, 95% CI 1.01-2.73) and stroke (HR 1.65, 95% CI 1.09-2.50) were significantly higher in patients with BD than in controls. Patients with BD also had a significantly higher risk of all-cause death (HR 1.82, 95% CI 1.40-2.37) compared to controls., Conclusion: Korean patients with BD had a higher overall risk of CVD than did those without BD. Therefore, patients with BD must be carefully monitored for the potential development of CVD to ensure that appropriate early treatments are delivered.