Treatment of Large Vessel Vasculitis

Abstract

Giant-cell arteritis (GCA) and Takayasus arteritis (TAK) are chronic and relapsing inflammatory diseases involving large and medium sized arteries. While symptoms derived from large-vessel involvement characterize TAK clinical presentation, cranial symptoms and complications usually dominate the clinical picture of GCA. However, delayed consequences of large-artery involvement are being increasingly recognized. Glucocorticoids are the mainstay of treatment for both conditions but flares and relapses are common when glucocorticoids are tapered or discontinued and adverse effects are frequent. Methotrexate has shown modest efficacy as glucocorticoid-sparing agent in GCA whereas infliximab did not demonstrate benefit in a randomized clinical trial. Adjuvant treatment for TAK is only supported by open-label trials and observational studies and small series, and methotrexate is the most widely used immunosuppressive agent. Infliximab shows promise for refractory/relapsing TAK as supported by an open label study with long-term follow up. Abatacept is currently being tested for both diseases in a randomized controlled trial. Other investigational agents in the horizon such as rituximab and tocilizumab have been anecdotally used but their efficacy needs to be confirmed. Revascularization procedures, mainly angioplasty, play a crucial role in the management of patients with TAK. Keywords: Giant-cell arteritis, Takayasu's disease, treatment, aortitis, aorta, Vasculitis, systemic vasculitis, revascularisation, biologic therapies