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  • Review Article
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Giant cell arteritis and polymyalgia rheumatica: current challenges and opportunities

Nature Reviews Rheumatology volume 13, pages 578–592 (2017)Cite this article

Subjects

Key Points

  • Giant cell arteritis (GCA) is best understood as an inflammatory vascular syndrome with features of cranial and/or large-vessel vasculitis, systemic inflammation and polymyalgia rheumatica (PMR), which frequently overlap

  • GCA and PMR are among the most common inflammatory rheumatic diseases in the elderly; the prevalence of these diseases is expected to increase due to ageing of the population

  • The role and value of imaging in GCA and PMR is evolving quickly

  • The pathophysiology of GCA is characterized by phases of initiation, transmural inflammation and chronic vessel wall injury and repair, each of which might be novel drug targets

  • Glucocorticoids are the standard-of-care treatment for GCA and PMR, although methotrexate is used in individual cases and anti-IL-6 therapy is now approved for the treatment of GCA

  • The selection of patients for biologic DMARD therapy, defining the best treatment strategies and the development of reliable outcome parameters are challenges in the future management of GCA and PMR

Abstract

The fields of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) have advanced rapidly, resulting in a new understanding of these diseases. Fast-track strategies and improved awareness programmes that prevent irreversible sight loss through early diagnosis and treatment are a notable advance. Ultrasonography and other imaging techniques have been introduced into routine clinical practice and there have been promising reports on the efficacy of biologic agents, particularly IL-6 antagonists such as tocilizumab, in treating these conditions. Along with these developments, which should improve outcomes in patients with GCA and PMR, new questions and unmet needs have emerged; future research should address which pathogenetic mechanisms contribute to the different phases and clinical phenotypes of GCA, what role imaging has in the early diagnosis and monitoring of GCA and PMR, and in which patients and phases of these diseases novel biologic drugs should be used. This article discusses the implications of recent developments in our understanding of GCA and PMR, as well as the unmet needs concerning epidemiology, pathogenesis, imaging and treatment of these diseases.

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Figure 1: Pathogenetic pathways and treatment targets in giant cell arteritis.

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Acknowledgements

We would like to thank K.S.M. van der Geest for help in preparing Figure 1 and C. Mackerness for administratively facilitating this Review.

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Authors and Affiliations

  1. Department of Rheumatology and Immunology, Medical University Graz, Auenbruggerplatz 15, Graz, 8036, Austria

    Christian Dejaco

  2. Rheumatology Service, South Tyrolean Health Trust, Hospital of Bruneck, Spitalstraße 11, Bruneck, 39031, Italy

    Christian Dejaco

  3. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9713, GZ, The Netherlands

    Elisabeth Brouwer

  4. Vascular Science and Rheumatology, Imperial College London, Guy Scadding Building, Cale Street, London, SW3 6LY, UK

    Justin C. Mason

  5. Department of Rheumatology and Clinical Immunology, Charité University Medicine, Chariteplatz 1, Berlin, D-10117, Germany

    Frank Buttgereit

  6. Division of Rheumatology and Division of Epidemiology, Departments of Internal Medicine and Health Sciences Research, Mayo Clinic College of Medicine and Science, 200 1st Street SW, Rochester, 55905, Minnesota, USA

    Eric L. Matteson

  7. Department of Rheumatology, Southend University Hospital and Anglia Ruskin University, Prittlewell Chase, SS0 ORY, Westcliff-on-sea, UK

    Bhaskar Dasgupta

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All authors made substantial contributions to discussions of the content, wrote the article and reviewed and/or edited the manuscript before submission.

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Correspondence to Bhaskar Dasgupta.

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Competing interests

C.D. declares that he has received consultancy fees and honoraria from AbbVie, Celgene, Lilly, Merck, MSD, Novartis, Pfizer, Roche, Sandoz and UCB, and unrestricted grant support from MSD and Pfizer, and has acted as a consultant and advisory board member for GSK. E.B. declares that she has received consultancy fees from Roche and an unrestricted grant from Janssen. J.M. declares that he has received consultancy fees and honoraria from Novartis and Roche. F.B. declares that he has received consultancy fees, honoraria and travel expenses from Galapagos, Horizon Pharma (formerly Nitec Pharma), Mundipharma and Roche and grant support from Horizon Pharma, and that he has served as co-principal investigator and site investigator in a Mundipharma-sponsored trial in PMR investigating the effects of modified-release prednisone. E.L.M. declares that he has served as coordinating investigator in a Novartis-sponsored PRM trial, as a consultant in a GSK-sponsored PMR trial, as a consultant for Endocyte and GSK and as a site investigator in GCA trials sponsored by Bristol Meyer Squibb, Genentech, GSK and Hoffman-LaRoche, and that he is an author and editor for UpToDate and Paradigm. B.D. declares that he has acted as a consultant and advisory board member for GSK, Merck, Mundipharma, Pfizer, Roche, Servier and Sobi) and that he has received unrestricted grant support from Napp and Roche and honoraria from Merck and UCB.

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Dejaco, C., Brouwer, E., Mason, J. et al. Giant cell arteritis and polymyalgia rheumatica: current challenges and opportunities. Nat Rev Rheumatol 13, 578–592 (2017). https://doi.org/10.1038/nrrheum.2017.142

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