ABSTRACT: OBJECTIVE:To assess the cardiovascular disease (CVD) and venous thromboembolism (VTE) risks among patients with newly diagnosed antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). PATIENTS AND METHODS:A population-based incident AAV cohort of 58 patients diagnosed between 1996 and 2015 in Olmsted County, MN, was identified by medical record review. For each patient, 3 age- and sex-matched non-AAV comparators were randomly selected from the same population and assigned an index date corresponding to the AAV incidence date. Medical records of cases and comparators were reviewed for CVD events, which included cardiac events (coronary artery disease, heart failure, and atrial fibrillation), cerebrovascular accidents (CVA), peripheral vascular disease (PVD), and VTE, which included deep vein thrombosis (DVT) and pulmonary embolism (PE). RESULTS:Baseline total cholesterol, high-density lipoprotein, and current smoking rate were lower in AAV than in comparators (P=.03, P=.01, and P=.04, respectively), whereas other CVD risk factors and Framingham risk score were not significantly different between the 2 groups. The CVD events developed in 13 patients and 17 comparators, corresponding to a more than 3-fold increased risk (hazard ratio [HR], 3.15; 95% CI, 1.51-6.57). By subtypes, risks were increased for cardiac events (HR, 2.96; 95% CI, 1.42-6.15) and CVA (HR, 8.16; 95% CI, 2.45-27.15), but not for PVD. The HR for VTE was 3.26 (95% CI, 0.84-12.60), significantly increased for DVT (HR, 6.25; 95% CI, 1.16-33.60), but not for PE (HR, 1.33; 95% CI, 0.23-7.54). CONCLUSION:Despite a similar prevalence of CVD risk factors at baseline, the risk of CVD is more than 3-fold higher and for CVA 8-fold higher in patients with incident AAV than in matched comparator subjects.