Large-vessel vasculitis

Pugh, D. et al. (2021) Large-vessel vasculitis. Nature Reviews Disease Primers, 7, 93. (doi: 10.1038/s41572-021-00327-5) (PMID:34992251)

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.

• Item Type: Articles
• Additional 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.
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Basu, Professor Neil and Goodyear, Professor Carl
• Authors: Pugh, D., Karabayas, M., Basu, N., Cid, M. C., Goel, R., Goodyear, C. S., Grayson, P. C., McAdoo, S. P., Mason, J. C., Owen, C., Weyand, C. M., Youngstein, T., and Dhaun, N.
• College/School: College of Medical Veterinary and Life Sciences > School of Infection & Immunity
• Research Centre: College of Medical Veterinary and Life Sciences > School of Infection & Immunity > Centre for Immunobiology
• Journal Name: Nature Reviews Disease Primers
• Publisher: Nature Research
• ISSN: 2056-676X
• ISSN (Online): 2056-676X
• Published Online: 06 January 2022
• Copyright Holders: Copyright © 2022 Springer Nature Limited
• First Published: First published in Nature Reviews Disease Primers 7: 93
• Publisher Policy: Reproduced in accordance with the publisher copyright policy