Large vessel vasculitis (LVV) is an inflammatory syndrome that primarily affects the aorta and its major branches, and includes Giant cell arteritis (GCA) and Takayasu arteritis (TA). Because it induces the ischemia of the peripheral organs and weakens the vascular wall, LVV is potentially a life-threatening condition; therefore, its differential diagnosis and optimal therapy should be introduced without delay. Although the mechanisms of LVV are not completely understood, by using diagnostic modalities such as CT and MRI, and sometimes FDG-PET, we may be able to diagnose LVV at a phase before prominent vascular destruction is manifest. IgG4-related periaortitis, a recently emerging clinical entity, may also appear as LVV. Similar to GCA and TA, IgG4-related disease has been specified as an intractable disease.
In this symposium, we would like to provide an update on our knowledge of LVVs, including epidemiology, cause, diagnosis, and treatment; and through which we can understand the whole picture of this relatively rare disorder.