Cardiovascular involvement in ANCA-associated vasculitis

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing vasculitis with significant cardiovascular involvement, primarily manifesting in granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. AAV patients face a 65% higher cardiovascular event risk, with common manifestations such as myocardial infarction, stroke and myocarditis, and less frequently, aortitis. Pathophysiology includes endothelial dysfunction, vascular inflammation, and direct inflammatory infiltration of cardiac tissue, exacerbated by traditional risk factors such as hypertension and dyslipidemia, and non-traditional factors like immunosuppressive therapies. Management emphasizes cardiovascular risk reduction through traditional methods alongside cautious use of glucocorticoids and immunosuppressants to control disease activity and minimize cardiovascular impact. Despite therapeutic advancements, long-term cardiovascular morbidity and mortality risks persist, underscoring the need for enhanced predictive tools and individualized therapeutic strategies. We aim to review current literature on the incidence, clinical manifestations and outcomes of cardiovascular involvement in AAV, with a focus on pathophysiology, risk factors, predictive models, and strategies for prevention and management.

Keywords: ANCA-associated vasculitis; cardiovascular mortality; immunosuppressive therapy; risk prediction; systemic vasculitis.