Journal Article

Giant cell arteritis and polymyalgia rheumatica: current challenges and opportunities

Mason, Justin C., Buttgereit, Frank, Brouwer, Elisabeth, Matteson, Eric L., Dejaco, Christian, Dasgupta, Bhaskar

2017

Overview

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 Rapid progress in the fields of giant cell arteritis and polymyalgia rheumatica has resulted in the introduction of imaging techniques into routine clinical practice and in promising reports on the efficacy of biologic agents for treatment. Further research should further advance our understanding of the epidemiology, pathogenesis, imaging and treatment of these diseases. 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.

• Publisher: Nature Publishing Group UK,Nature Publishing Group
• Subject: 59/57 / 59/78 / 692/4023/1670/595 / 692/420/2780 / 692/53 / 692/700/1421 / Antagonists / Antibodies, Monoclonal, Humanized - therapeutic use / Arteritis / Autoimmune diseases / Care and treatment / Clinical Trials as Topic / Development and progression / Diagnosis / Early Diagnosis / Epidemiology / Giant Cell Arteritis - diagnostic imaging / Giant Cell Arteritis - drug therapy / Giant Cell Arteritis - epidemiology / Humans / Interleukin 6 / Medical research / Medicine & Public Health / Patients / Phenotypes / Polymyalgia rheumatica / Polymyalgia Rheumatica - diagnostic imaging / Polymyalgia Rheumatica - drug therapy / Polymyalgia Rheumatica - epidemiology / review-article / Rheumatology / Temporal arteritis / Ultrasound / Vein & artery diseases