Takayasu’s arteritis: a cell-mediated large-vessel vasculitis

Abstract

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.

Hemiballism with insular infarction as first manifestation of Takayasu's arteritis in association with chronic hepatitis B

“…Clinically the disease presents with central nervous symptoms, cardiac problems (heart failure, arrhythmia, infarction), renal failure or aneurysmal dissection or rupture. Cell-mediated autoimmunity leading to vascular injury has been suspected in its pathogenesis although the antigen inducing the process remains unknown [11]. We describe a case of Takayasu's arteritis with an unusual clinical presentation and discuss the role of chronic hepatitis B infection for the development of arteritis.…”

“…The etiology of Takayasu's arteritis has been related to a combination of genetic predisposition, antigen-specific cellular immune responses and T-cell-activation leading to progressive fibrosis [11]. Cell-mediated autoimmunity leading to vascular injury has been suspected in the pathogenesis of Takayasu's arteritis although the antigen inducing the process remains unknown.…”

“…Showing varying degrees of severity and activity it remains a potentially life-threatening disease with mortality ranging up to 35 % over 5 years [3]. Clinically the disease presents with central nervous symptoms, cardiac problems, renal failure or aneurysmal dissection or rupture [11].…”

Scleritis and Takayasu’s disease – rare combined presentation

“…The 5 years mortality of TA being 35%, (Rizzi et al 1999) it is an important diagnosis which may often be apparent after thorough history and examination. Although rare, we document an association between TD and scleritis.…”

Aortic regurgitation and aortic valve operation in Takayasu arteritis: A meta-analysis

“…[2] Although TA has a worldwide distribution, the greatest prevalence is seen in Asian countries. [3] The diagnosis of TA can sometimes be difficult, particularly in patients with non-specific symptoms. Thus, a delayed or missed diagnosis until after the occurrence of a major complication is frequent.…”