Recent advances in the treatment of giant cell arteritis

Abstract

Giant cell arteritis (GCA) is a systemic, granulomatous, large-vessel vasculitis that affects individuals over the age of 50 years. Morbidity from disease includes cranial manifestations which can cause irreversible blindness, while extra-cranial manifestations can cause vascular damage with large-artery stenosis, occlusions, aortitis, aneurysms, and dissections. Glucocorticoids while efficacious are associated with significant adverse effects. Furthermore, despite treatment with glucocorticoids, relapses are common. An understanding of the pathogenesis of GCA has led to the discovery of tocilizumab as an efficacious steroid-sparing therapy while additional therapeutic targets affecting different inflammatory pathways are under investigation. Surgical treatment may be indicated in cases of refractory ischemia or aortic complications but data on surgical outcomes are limited. Despite the recent advances, many unmet needs exist, including the identification of patients or subsets of GCA who would benefit from earlier initiation of adjunctive therapies, patients who may warrant long-term immunosuppression and medications that sustain permanent remission. The impact of medications like tocilizumab on long-term outcomes, including the development of aortic aneurysms and vascular damage also warrants investigation.

Keywords: Biologic therapies; Giant cell arteritis; Glucocorticoids; Guidelines; Large-vessel vasculitis; Leflunomide; Methotrexate; Recommendations; Tocilizumab; Treatment.