Current and Emerging Therapies in Large-Vessel Vasculitis

Tanaz A. Kermani; Bhaskar Dasgupta


Rheumatology. 2018;57(9):1513-1524. 

In This Article

Abstract and Introduction


GCA shares many clinical features with PMR and Takayasu arteritis. The current mainstay of therapy for all three conditions is glucocorticoid therapy. Given the chronic, relapsing nature of these conditions and the morbidity associated with glucocorticoid therapy, there is a need for better treatment options to induce and sustain remission with fewer adverse effects. Conventional immunosuppressive treatments have been studied and have a modest effect. There is a keen interest in biologic therapies with studies showing the efficacy of IL-6 antagonists in PMR and GCA. Recently the first two randomized clinical trials in Takayasu arteritis have been completed. A major challenge for all of these conditions is the lack of standardized measures to assess disease activity. Long-term studies are needed to evaluate the impact of biologic therapies showing potential on important clinical outcomes such as vascular damage, cost-effectiveness and quality of life. The optimal duration of treatment also needs to be assessed.


Large-vessel vasculitis (LVV), which includes GCA and Takayasu arteritis (TAK), affects the aorta and its branches.[1] GCA and TAK share similarities, including clinical, radiographic and histopathologic features, but also important differences.[1–4] PMR is a common inflammatory condition that shares many similarities and associations with GCA[5–7] and may be part of the same disease spectrum.

GCA, TAK and PMR are responsive to glucocorticoid (GC) therapy, which has been considered the mainstay. However, these are chronic relapsing conditions, and prolonged GC therapy is associated with significant morbidity.[8–12] Conventional immunosuppressants have modest GC-sparing effect. There is an urgent unmet need for DMARDs such as LEF in these conditions. With the advent of biologic therapies, there has been an interest in targeted therapies. In this review we discuss alternatives to GC treatment with an emphasis on biologic therapies.