Large Vessel Vasculitis

Dan Pugh, Maira Karabayas, Neil Basu, Maria C Cid, Ruchika Goel, Carl S Goodyear, Peter C. Grayson, Stephen P McAdoo, Justin C Mason, Catherine Owen , Cornelia Weyand, Taryn Youngstein, Neeraj Dhaun

Research output: Contribution to journalArticlepeer-review

Abstract

Large vessel vasculitis (LVV) manifests as inflammation of the aorta and its major branches, and is the most common primary vasculitis in adults. Although comprised of only two distinct conditions, giant cell arteritis (GCA) and Takayasu arteritis (TAK), the phenotypic spectrum of primary LVV is complex. Non-specific symptoms often predominate and so patients with LVV may present to different healthcare providers and settings. Rapid diagnosis, specialist referral and early treatment are key to good patient outcomes. Unfortunately, disease relapse remains common and chronic vascular complications are a source of significant morbidity. The ability to accurately monitor disease activity remains challenging and recent progress in both vascular imaging techniques and laboratory biomarkers may facilitate better matching of treatment intensity with disease activity. Advances in pathophysiological understanding have paved the way for novel biologic treatments which target important mediators of disease in both GCA and TAK. Such work has highlighted the significant heterogeneity present within LVV and the importance of an individualized therapeutic approach. Future work will focus on understanding the mechanisms behind persisting vascular inflammation which will inform the development of increasingly sophisticated imaging technology. Together, these will allow better disease prognostication, limit treatment-associated adverse effects, and facilitate the development and use of novel therapies in a more targeted way.

Original languageEnglish
JournalNature Reviews Disease Primers
DOIs
Publication statusPublished - 6 Jan 2022

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