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Large-vessel vasculitis

Nature Reviews Disease Primers volume 7, Article number: 93 (2021) Cite this article

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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.

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Fig. 1: Disease classification and arterial involvement in LVV.
Fig. 2: Global incidence of large-vessel vasculitis.
Fig. 3: Proposed factors contributing to a loss of immune tolerance of large arteries and initiation of inflammation in LVV.
Fig. 4: Mediators of inflammation in large-vessel vasculitis.
Fig. 5: Investigation and diagnosis of LVV.
Fig. 6: Longitudinal follow-up imaging using FDG-PET.
Fig. 7: PET–MRI in large-vessel vasculitis.
Fig. 8: Management of LVV.

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Acknowledgements

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.

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Authors and Affiliations

  1. British Hearth Foundation/University Centre for Cardiovascular Science, University of Edinburgh, Edinburgh, UK

    Dan Pugh & Neeraj Dhaun

  2. Centre for Arthritis & Musculoskeletal Health, University of Aberdeen, Aberdeen, UK

    Maira Karabayas

  3. Institute of Infection, Immunity & Inflammation, University of Glasgow, Glasgow, UK

    Neil Basu & Carl S. Goodyear

  4. Department of Autoimmune Diseases, Institut d’Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain

    Maria C. Cid

  5. Department of Clinical Immunology & Rheumatology, Christian Medical College, Vellore, India

    Ruchika Goel

  6. National Institute of Arthritis & Musculoskeletal & Skin Diseases, National Institutes of Health, Bethesda, MA, USA

    Peter C. Grayson

  7. Department of Immunology & Inflammation, Imperial College London, London, UK

    Stephen P. McAdoo

  8. National Heart & Lung Institute, Imperial College London, London, UK

    Justin C. Mason & Taryn Youngstein

  9. Patient representative, Edinburgh, UK

    Catherine Owen

  10. Centre for Translational Medicine, Stanford University, Stanford, California, USA

    Cornelia M. Weyand

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Contributions

Introduction (N.D., D.P., J.C.M. and T.Y.); Epidemiology (N.D., D.P., R.G., S.P.M. and T.Y.); Mechanisms/pathophysiology (N.D., D.P., M.K., C.S.G., C.M.W. and T.Y.); Diagnosis/screening/prevention (N.D., D.P., P.C.G. and T.Y.); Management (N.D., D.P., N.B., M.C.C. and T.Y.); Quality of life (N.D., D.P., R.G., S.P.M., C.O. and T.Y.); Outlook (N.D., D.P., J.C.M. and T.Y.); Overview of the Primer (N.D., D.P., J.C.M. and T.Y.).

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Correspondence to Neeraj Dhaun.

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Nature Reviews Disease Primers thanks Christian Dejaco, who co-reviewed with Milena Bond; David Saadoun; Maxime Samson; and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Pugh, D., Karabayas, M., Basu, N. et al. Large-vessel vasculitis. Nat Rev Dis Primers 7, 93 (2021). https://doi.org/10.1038/s41572-021-00327-5

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