Update on the Epidemiology and Treatment of Giant Cell Arteritis

Abstract

Giant cell arteritis is the most common form of primary systemic vasculitis in people over the age of 50 years. This disease affects the aorta and its branches, and complications may include vision loss, arterial stenosis, and aortic aneurysm. Early recognition and treatment of giant cell arteritis is important to mitigate long-term damage. While glucocorticoids remain the mainstay of treatment, relapses are common and morbidity related to treatment frequently occurs. There is an unmet need for immunosuppressive therapies that are able to induce long-term remission while avoiding the adverse effects of glucocorticoids. Of available conventional immunosuppressive treatments, methotrexate is generally considered a first-line steroid-sparing agent for patients with disease refractory to glucocorticoids and in those at high risk for glucocorticoid-associated toxicity. Advances in understanding of disease pathogenesis continue to identify potential therapeutic targets, and several biologic agents are currently under investigation. Preliminary reports from controlled studies demonstrate potential efficacy of tocilizumab and abatacept in both newly diagnosed and relapsing patients; however, use of these agents should not yet be considered standard therapy. The role of anti-platelet therapy in prevention of ischemic complications is controversial, but low-dose aspirin is considered reasonable in this population. Statin medications have little role in the management of giant cell arteritis (GCA) and should be reserved for those with clinical indications for lipid reduction. Correction of arterial stenosis with surgical revascularization is uncommonly required for patients with refractory ischemic symptoms. Intervention should be performed in patients with quiescent disease. Monitoring for late-onset complications, particularly thoracic aortic aneurysm, should take place though the frequency of screening and the optimal timing of intervention is not established.