Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare, multisystem, autoimmune disease characterised by microvascular inflammation. Over the past 20 years, advances in immunological management have improved short-term patient outcomes. Longer-term, patient outcomes remain poor with cardiovascular disease now the leading cause of death in AAV. Importantly, the incidence of cardiovascular disease in these patients exceeds that expected by traditional risk factors alone, suggesting a contribution from disease-specific factors. There are currently no validated tools that accurately assess cardiovascular disease risk in patients with AAV. Similarly, it is unclear how different immunosuppressive therapies contribute to and modify cardiovascular risk, and there is a paucity of data examining the efficacy of traditional cardioprotective medications in AAV. In this review we examine the potential pathways that contribute to the increased risk of cardiovascular disease in AAV, and the current evidence to manage this risk
