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The primary systemic vasculitides are a group of autoimmune conditions characterised by occlusion, stenosis or aneurysmal dilatation of blood vessels secondary to intra-mural inflammation. Current therapy has converted the outlook of these diseases from death or severe morbidity to a remitting-relapsing condition in most instances. Longer survival, relapsing course of disease and chronic glucocorticoid therapy probably contribute to an increase in cardiovascular events and morbidity. This article reviews the available data for effect of primary systemic vasculitis on cardiovascular end points like coronary artery disease, congestive cardiac failure, hypertension and aortic aneurysm in all age groups. We examine the interplay between the activated endothelium, autoimmune mechanisms and treatment factors to produce a direct insult or increased atherogenic potential of primary systemic vasculitis. Recommendations to deal with cardiovascular end points are made.

Original publication




Journal article


Best pract res clin rheumatol

Publication Date





419 - 428


Adolescent, Aortic Aneurysm, Cardiovascular Diseases, Child, Child, Preschool, Coronary Artery Disease, Female, Heart Failure, Humans, Hypertension, Male, Survival Rate, Vasculitis, Young Adult