Takayasu's arteritis is an idiopathic, systemic inflammatory disease, typically involving the aorta and its main branches. Cell-mediated autoimmunity has been strongly implicated in its pathogenesis. Early or active-stage pathology consists of continuous or patchy granulomatous inflammation, which progresses to intimal and adventitial fibrosis and scarring of the media. Multiple focal or segmental stenoses result and aneurysms may occasionally occur. Clinical presentation is heterogeneous, ranging from asymptomatic to catastrophic. In some patients, constitutional signs and symptoms indicating a systemic inflammatory response are observed, usually in the early stages. Specific features reflect arterial involvement, and result from end-organ or limb ischemia; they include vascular, neurological, cardiac, and pulmonary manifestations. The course of Takayasu's arteritis usually extends for many years with varying degrees of activity. Takayasu's arteritis has a worldwide distribution, with the greatest prevalence in eastern countries. Women of reproductive age are preferentially affected, but the illness is being recognized with increasing frequency in males. Variable phenotypes are recently emerging in different ethnic groups. Diagnosis is based on clinical features and vascular imaging studies that document typical patterns of stenoses or aneurysms of the aorta and its primary branches. Assessment of the activity of Takayasu's arteritis is imprecise, in that clinical features and acute-phase reactants do not accurately reflect active blood vessel inflammation. High-dose corticosteroids alone or a cytotoxic agent in addition to a corticosteroid may be effective in treating active disease. Critical lesions may require correction by surgery or interventional radiology.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.