Idiopathic Non-atherosclerotic Carotid Artery Disease

Purpose of review The purpose of this review is to provide an overview of idiopathic non-atherosclerotic causes of carotid artery disease and its manifestations. Recent findings Four major causes of non-atherosclerotic carotid artery disease including dissection, fibromuscular dysplasia, moyamoya disease, and inflammatory large vessel vasculitis are discussed. While there is a dearth of clinical trials involving some of the rarer conditions, recent data from clinical trials supporting antiplatelet over anticoagulation treatment of cervical artery dissection, recent consensus statements on the management of fibromuscular dysplasia, and guideline approaches to diagnosis and treatment of large vessel vasculitis are summarized. Idiopathic non-atherosclerotic causes of carotid artery disease are under appreciated and may lead to significant morbidity and mortality. While less common compared with atherosclerotic disease, non-atherosclerotic disease may affect younger patient populations and result in non-cerebrovascular arterial involvement and systemic organ damage. Therefore, prompt recognition of these disorders is key to their management.