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  • Review Article
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Monitoring and long-term management of giant cell arteritis and polymyalgia rheumatica

Nature Reviews Rheumatology volume 16, pages 481–495 (2020)Cite this article

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An Author Correction to this article was published on 10 September 2020

This article has been updated

Abstract

Giant cell arteritis (GCA) is the most common type of primary vasculitis in Western countries. Polymyalgia rheumatica (PMR) is the second most common inflammatory rheumatic disease of the elderly after rheumatoid arthritis. Glucocorticoids are the cornerstone of treatment for GCA and PMR, which are interrelated diseases. Glucocorticoids are effective, but adverse effects occur in a high proportion of patients. Careful use of glucocorticoids and the application of preventive strategies can minimize these adverse effects. Possible long-term complications of GCA include aneurysm and stenosis of vessels, even in patients with apparently clinically inactive disease; acute blindness is rare during glucocorticoid treatment. In PMR, whether subclinical chronic inflammation can lead to long-term damage is less clear. Management of both GCA and PMR is hampered by the lack of universally accepted definitions of remission and other disease states, such as low disease activity or vessel damage without active disease. In this Review, we outline current evidence on the monitoring and long-term management of patients with GCA and PMR, including the tapering of treatment.

Key points

  • Management of both giant cell arteritis (GCA) and polymyalgia rheumatica a (PMR) is hampered by the lack of universally accepted definitions of remission and other disease states, such as low disease activity or vessel damage without active disease.

  • Long-term management of GCA and PMR should be tailored to individual patient characteristics, including disease manifestations, as well as risk factors for treatment and disease-related complications.

  • Imaging might help clinicians to monitor disease activity and damage; however, which imaging techniques to use and when to use them remains unclear.

  • Although novel therapies such as anti-IL-6 agents help to reduce glucocorticoid exposure and achieve drug-free remission, questions remain about the best treatment regimens and biomarkers to monitor disease activity and predict flare after discontinuation of treatment.

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Fig. 1: Imaging of chronic large-vessel vasculitis during glucocorticoid treatment.
Fig. 2: Common clinical scenarios, and their management, in the long-term follow-up of patients with GCA and PMR.

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Change history

  • 10 September 2020

    An amendment to this paper has been published and can be accessed via a link at the top of the paper.

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Authors and Affiliations

  1. Division of Rheumatology, Local Health Trust 3, Genoa, Italy

    Dario Camellino

  2. Autoimmunology Laboratory, University of Genoa, Genoa, Italy

    Dario Camellino

  3. Division of Rheumatology, and Division of Epidemiology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA

    Eric L. Matteson

  4. Charité University Medicine, Department of Rheumatology and Clinical Immunology, Berlin, Germany

    Frank Buttgereit

  5. Medical University Graz, Department of Rheumatology and Immunology, Graz, Austria

    Christian Dejaco

  6. Hospital of Brunico (SABES-ASDAA), Department of Rheumatology, Brunico, Italy

    Christian Dejaco

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D.C. and C.D. researched data for the article. D.C., E.L.M., F.B. and C.D. wrote the article. All authors made a substantial contribution to discussion of content and reviewed or edited the manuscript before submission.

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D.C. has received consultancy and speaker fees from AbbVie, Celgene, Janssen-Cilag, Lilly, Mylan, Novartis and Sanofi. E.L.M. was a site investigator for the GiACTA study and is a contributor and section editor for UpToDate. F.B. has received consultancy and speaker fees as well as research grants from Mundipharma, Horizon and Roche/Chugai. C.D. has received consultancy and speaker fees from AbbVie, BMS, Lilly, MSD, Novartis, Pfizer, Roche, Sanofi and UCB, and a research grant from Celgene.

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Glossary

Claudication

Cramping pain, elicited by repetitive activities (such as chewing or walking), owing to reduced blood flow.

Vascular bruits

An abnormal sound made on the auscultation of an artery, owing to a turbulent flow secondary to vascular stenosis.

Girdles

The parts of the appendicular skeleton that anchor the limbs to the axial skeleton, even though they are broadly defined as the shoulder and pelvis areas.

Amaurosis fugax

Transient visual loss (from the ancient Greek “ᾰμαύρωσῐς”, meaning darkening, and the Latin “fugax”, meaning brief, fleeting).

Diplopia

The vision of two images of a single object (also called double vision).

Remitting seronegative symmetrical synovitis with pitting oedema

(RS3PE). A clinical syndrome characterized by bilateral, diffuse swelling and inflammation of the hands and/or feet.

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Camellino, D., Matteson, E.L., Buttgereit, F. et al. Monitoring and long-term management of giant cell arteritis and polymyalgia rheumatica. Nat Rev Rheumatol 16, 481–495 (2020). https://doi.org/10.1038/s41584-020-0458-5

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