Clinical features and diagnosis of Takayasu arteritis
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
Takayasu arteritis (TAK) is classified as a large-vessel vasculitis because it primarily affects the aorta and its primary branches. It also shares some histologic and clinical features with giant cell (temporal) arteritis (GCA), the other major large-vessel vasculitis. Patients may present initially with constitutional symptoms but later develop symptoms associated with vascular damage.
The pathogenesis, pathology, clinical manifestations, and diagnosis of TAK will be reviewed here. The treatment of this disorder is discussed separately (see "Treatment of Takayasu arteritis"). Overviews of the vasculitides in children and in adults are also discussed elsewhere. (See "Vasculitis in children: Classification and incidence" and "Overview of and approach to the vasculitides in adults".)
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 Asia [3-5]. In Japan, it has been estimated that 150 new cases occur each year ; by comparison, the incidence is one to three new cases per year per million population in the United States and Europe .
The pathogenesis of Takayasu arteritis (TAK) is poorly understood. Cell-mediated mechanisms are thought to be of primary importance and may be similar to those in giant cell arteritis (GCA) . (See "Pathogenesis of giant cell (temporal) arteritis".)
Immunohistopathologic examination has shown that the infiltrating cells in aortic tissue mainly consist of killer cells, especially gamma delta T lymphocytes . These cells may cause vascular injury by releasing large amounts of the cytolytic compound perforin. Recognition of heat shock protein-65 might facilitate recognition and adhesion of the infiltrating cells. In another report, the T cell receptors on the infiltrating T cells had a restricted repertoire, suggesting that a specific but as yet unidentified antigen in aortic tissue might be targeted . This change was not seen in atherosclerotic aortic aneurysms.
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