Review
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 16, 2015; 3(6): 484-494
Published online Jun 16, 2015. doi: 10.12998/wjcc.v3.i6.484
Giant cell arteritis: Current treatment and management
Cristina Ponte, Ana Filipa Rodrigues, Lorraine O’Neill, Raashid Ahmed Luqmani
Cristina Ponte, Ana Filipa Rodrigues, Lorraine O’Neill, Raashid Ahmed Luqmani, Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, Oxford OX1 2JD, United Kingdom
Cristina Ponte, Department of Rheumatology, Hospital de Santa Maria, CHLN, Lisbon Academic Medical Centre, 1649-035 Lisbon, Portugal
Ana Filipa Rodrigues, Department of Internal Medicine, Hospital das Caldas da Rainha, Centro Hospitalar Oeste, 2500-176 Caldas da Rainha, Portugal
Lorraine O’Neill, Department of Rheumatology, St Vincent’s University Hospital, Dublin, Ireland
Author contributions: All authors contributed to the writing of this review manuscript.
Conflict-of-interest: The authors have no conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Cristina Ponte, MD, Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, Nuffield Orthopaedic Centre, Windmill Road, Oxford OX3 7LD, United Kingdom. cristinadbponte@gmail.com
Telephone: +44-1865-227374
Received: January 5, 2015
Peer-review started: January 20, 2015
First decision: January 30, 2015
Revised: February 28, 2015
Accepted: March 30, 2015
Article in press: April 2, 2015
Published online: June 16, 2015
Processing time: 165 Days and 13.1 Hours
Abstract

Glucocorticoids remain the cornerstone of medical therapy in giant cell arteritis (GCA) and should be started immediately to prevent severe consequences of the disease, such as blindness. However, glucocorticoid therapy leads to significant toxicity in over 80% of the patients. Various steroid-sparing agents have been tried, but robust scientific evidence of their efficacy and safety is still lacking. Tocilizumab, a monoclonal IL-6 receptor blocker, has shown promising results in a number of case series and is now being tested in a multi-centre randomized controlled trial. Other targeted treatments, such as the use of abatacept, are also now under investigation in GCA. The need for surgical treatment is rare and should ideally be performed in a quiescent phase of the disease. Not all patients follow the same course, but there are no valid biomarkers to assess therapy response. Monitoring of disease progress still relies on assessing clinical features and measuring inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Imaging techniques (e.g., ultrasound) are clearly important screening tools for aortic aneurysms and assessing patients with large-vessel involvement, but may also have an important role as biomarkers of disease activity over time or in response to therapy. Although GCA is the most common form of primary vasculitis, the optimal strategies for treatment and monitoring remain uncertain.

Keywords: Giant cell arteritis; Therapy; Disease management; Glucocorticoids; Immunosuppressive agents

Core tip: Giant cell arteritis (GCA) is the most common form of primary systemic vasculitis. Treatment with high doses of glucocorticoids should be initiated as early as possible to prevent ischaemic manifestations, such as blindness (occurring in up to 20%). However, glucocorticoid therapy leads to significant toxicity in over 80% of the patients. Various steroid-sparing agents have been tried, but robust scientific evidence of their efficacy and safety is still lacking. Not all patients follow the same course, but there are no valid biomarkers to assess therapy response. The authors review the optimal strategies for treatment and monitoring of patients with GCA.