[Large vessel vasculitis]

Abstract

Giant cell arteritis (GCA) and Takayasu arteritis (TA) are the two diseases characterized as large vessel vasculitis (LVV) and are autoimmune diseases with granulomatous inflammation that affect medium and large sized arteries. These diseases are accompanied by symptoms of systemic inflammatory reactions typically including fatigue, weight loss and low grade fever as well as elevation of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels. More specific symptoms include headache and visual symptoms for GCA and arm or leg claudication, renal hypertension and angina pectoris for TA. Imaging studies to demonstrate inflammatory vascular wall lesions and biopsy of the temporal artery for GCA are the most relevant diagnostic procedures. Treatment relies mainly on glucocorticoids. Methotrexate seems to have a moderate glucocorticoid-sparing effect but evidence for other immunosuppressants, including azathioprine, tocilizumab and cyclophosphamide is limited. Revascularization methods might also be required in TA.