Takayasu Disease

This is a larger-vessel granulomatous vasculitis with particular involvement of the aorta and its major branches. It is typically seen in patients less than 40 years of age [54]. It is much more commonly seen in women than men. Aortic imaging in patients suffering from large artery occlusive disease, with loss of pulses (pulseless disease), is vital in diagnostic evaluation. Systemic manifestations can precede occlusive events and include fever, malaise, and loss of appetite. Elevated ESR and CRP call attention to the inflammatory nature of this rare disorder. The natural history can range from mild to severe [75]. Involvement of the carotid arteries can result in ischemic optic neuropathy and stroke. Corticosteroid therapy is traditionally the first line of immunosuppression. Unfortunately, the ongoing need for such therapy often leads to side effects. Agents used for steroid sparing include azathio- prine, methotrexate, antitumor necrosis factor receptor agents, rituximab, and cyclophosphamide with some degree of response reported in open-label trials [76].

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