Large-vessel vasculitis.

Abstract

Primary large-vessel vasculitis encompasses conditions that, despite sharing many common features, constitute distinct entities that have their own prognostic implications. These conditions include giant cell arteritis and Takayasu arteritis, with isolated aortitis being increasingly recognised in the literature and studied within this disease spectrum. Epidemiological studies have evidenced a worldwide distribution of Takayasu arteritis. In giant cell arteritis, distinct clinical phenotypes with specific outcomes (ie, cranial and large vessel forms) have been recognised. The advancements that have been made in vascular imaging have enabled improvement in diagnosis and classification of these diseases, although their value in follow-up continues to be assessed. Targeted therapies that can induce clinical remission with reduced glucocorticoid exposure are emerging. However, many patients develop vascular damage over time, highlighting the need for further understanding of the pathophysiological link between inflammation, vascular injury, and remodelling.