40 - Large Vessel Vasculitis

Abstract

Large vessel vasculitis refers to a group of diseases involving inflammation of the aorta and/or its major large arterial branches not caused by infection, radiation, or trauma. These diseases may come to the attention of vascular medicine specialists through any one of the multiple clinical manifestations of disease the disorders cause, including claudication, limb ischemia, stroke, visual loss, cardiac disease, hypertension, and aortic aneurysms. Large vessel vasculitis may also be suspected only after arterial changes are found incidentally through imaging studies. This chapter focuses on the two main forms of large vessel vasculitis, Takayasu arteritis and giant cell arteritis, as well as isolated aortitis. Large vessel vasculitis can also rarely be seen in other forms of vasculitis or systemic rheumatic disease such as Behçet disease, Cogan syndrome, immunoglobulin G4–related disease, relapsing polychondritis, or spondyloarthropathy.