Despite improved prognosis, patients with systemic lupus erythematosus (SLE) remain at increased risk for early death. Vascular events (VE) occur with increased frequency and contribute to premature death in SLE patients. As conventional cardiovascular risk factors do not fully explain this hazard, this study investigated the contribution of disease-specific features to VE development. Documented VE were classified as atherothrombotic, venous thrombotic, arterial thrombotic or tissue loss inducing vasculitis during a mean follow-up of nearly 12 years in the Tromsø Lupus cohort (n = 158). The impact of disease-specific factors (organ manifestations, laboratory findings, drug treatment, weighted average SLE Disease Activity Index (WAS) and cumulative Damage Index) was assessed by odds ratios for VE in multivariate analysis. A total of 41 patients (26%) developed VE, and atherothrombotic events were most common (73%). Overall, VE prevalence was 3.5/100 patient years, and VE risk increased linearly over time, reaching 35% after 20 years. WAS scores >3 increased, and use of hydroxychloroquine and antihypertensive medication reduced overall VE risk. Age >40 years was the main risk factor for atherothrombotic events. VE nearly quadrupled the risk of death. VE occurred in 26% of SLE patients, predominantly as atherothrombotic disease. VE prevalence increased linearly over time leading to a four-fold risk of mortality. Strategies for reducing disease activity, including treatment with antimalarials and antihypertensive drugs, are most likely to reduce the risk associated with VE in SLE.