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Clinical features and diagnosis of Takayasu arteritis

Author
Gene G Hunder, MD
Section Editor
Eric L Matteson, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH

INTRODUCTION

Takayasu arteritis is a chronic vasculitis of unknown etiology. Women are affected in 80 to 90 percent of cases, with an age of onset that is usually between 10 and 40 years [1,2]. It has a worldwide distribution, with the greatest prevalence in Asians [3-5]. In Japan, it has been estimated that 150 new cases occur each year [6]; by comparison, the incidence is one to three new cases per year per million population in the United States and Europe [2]. Human leukocyte antigen (HLA)-Bw52 and HLA-B39.2 have been increased in frequency in several studies, suggesting an immunogenetic association [7].

Takayasu arteritis primarily affects the aorta and its primary branches [8]. The inflammation may be localized to a portion of the thoracic or abdominal aorta and branches, or may involve the entire vessel. Although there is considerable variability in disease expression (due perhaps to geographic differences [9]), the initial vascular lesions frequently occur in the left middle or proximal subclavian artery. As the disease progresses, the left common carotid, vertebral, brachiocephalic, right middle or proximal subclavian artery, right carotid, vertebral arteries, and aorta may also be affected. The abdominal aorta and pulmonary arteries are involved in approximately 50 percent of patients. (See "Overview of and approach to the vasculitides in adults".)

The inflammatory processes cause thickening of the walls of the affected arteries. The proximal aorta may become dilated secondary to inflammatory injury. Narrowing, occlusion, or dilation of involved portions of the arteries in varying degrees results in a wide variety of symptoms [10].

The pathogenesis, pathology, clinical manifestations, and diagnosis of Takayasu arteritis will be reviewed here. The treatment of this disorder is discussed separately. (See "Treatment of Takayasu arteritis".)

PATHOGENESIS

The pathogenesis of Takayasu arteritis is poorly understood. Cell-mediated mechanisms are thought to be of primary importance and may be similar to those in giant cell (temporal) arteritis [11]. (See "Pathogenesis of giant cell (temporal) arteritis".)

                   

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Literature review current through: Nov 2016. | This topic last updated: Thu Nov 06 00:00:00 GMT 2014.
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