Emerging pharmacotherapies for large-vessel vasculitis.

Large-vessel vasculitis (LVV) constitutes a heterogeneous group of diseases including giant cell arteritis (GCA), Takayasu's arteritis (TAK), isolated aortitis, and the aortitis associated with other systemic autoimmune or inflammatory diseases. This narrative review shows briefly the main epidemiological and clinical features of LVV and discusses in depth both the classic and the new therapeutic options used in LVV. With this objective, a structure literature search of the main original manuscripts and reviews published in English in major medical databases, including PubMed, EMBASE, and Cochrane Library from 2016 to now was conducted. Treatment should always follow a T2T strategy, aimed at reaching clinical remission. In general, patients with LVV experience a rapid response to high doses of glucocorticoids, but relapses frequently occur when the dose is reduced or discontinued. Tocilizumab has been successfully used as a glucocorticoid-sparing agent in both GCA and TAK. Besides, JAK inhibitors have demonstrated efficacy in LVV, both inducing remission and reducing glucocorticoid doses. Apart from these drugs, no other agents have yet been approved for the treatment of these conditions. This review examines classic and new therapeutic options with greatest potential for the future treatment of LVV.