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 M Weyand, Taryn Youngstein, Neeraj Dhaun

Research output: Contribution to journalArticlepeer-review

85 Citations (Scopus)

Abstract

Large-vessel vasculitis (LVV) manifests as inflammation of the aorta and its major branches and is the most common primary vasculitis in adults. LVV comprises two distinct conditions, giant cell arteritis and Takayasu arteritis, although the phenotypic spectrum of primary LVV is complex. Non-specific symptoms often predominate and so patients with LVV present to a range of health-care 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 considerable morbidity. Although accurate monitoring of disease activity is challenging, progress in vascular imaging techniques and the measurement of laboratory biomarkers may facilitate better matching of treatment intensity with disease activity. Further, advances in our understanding of disease pathophysiology have paved the way for novel biologic treatments that target important mediators of disease in both giant cell arteritis and Takayasu arteritis. This work has highlighted the substantial heterogeneity present within LVV and the importance of an individualized therapeutic approach. Future work will focus on understanding the mechanisms of persisting vascular inflammation, which will inform the development of increasingly sophisticated imaging technologies. Together, these will enable better disease prognostication, limit treatment-associated adverse effects, and facilitate targeted development and use of novel therapies.

Original languageEnglish
Article number93
Pages (from-to)93
Number of pages23
JournalNature reviews. Disease primers
Volume7
Issue number1
DOIs
Publication statusPublished - 6 Jan 2022

Bibliographical note

© 2022. Springer Nature Limited.

Funding Information:
D.P. and M.K. are funded by Clinical Academic Fellowships from the Chief Scientist Office, Scotland (CAF/19/01 and CAF/21/05, respectively). M.C.C. is funded by the Ministerio de Ciencia e Innovación/AEI//10.13039/501100011033 (PID2020-114909RB-I00) and the International Vasculitis Foundation. N.D. is supported by a Senior Clinical Research Fellowship from the Chief Scientist Office (SCAF/19/02). J.C.M., S.P.M. and T.Y. acknowledge infrastructure support from the Imperial National Institute for Health Research (NIHR) Biomedical Research Centre. P.C.G. is supported by the Intramural Research Program at the National Institute of Arthritis and Musculoskeletal and Skin Diseases.

Publisher Copyright:
© 2022, Springer Nature Limited.

Keywords

  • Adult
  • Aorta
  • Giant Cell Arteritis/complications
  • Humans
  • Takayasu Arteritis/complications

Fingerprint

Dive into the research topics of 'Large-vessel vasculitis'. Together they form a unique fingerprint.

Cite this