This article includes discussion of Takayasu arteritis, pulseless disease, Takayasu disease, and Takayasu syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Takayasu arteritis, sometimes called “pulseless disease,” is a rare form of vasculitis affecting the aorta and its major branches, resulting in large-vessel occlusions. It typically occurs in young Asian women, but can be found in any ethnic group and in men. The disease is heterogeneous in presentation. Early symptoms are often constitutional, making diagnosis difficult. Subsequently, arterial occlusions occur, producing more specific ischemic symptoms such as claudication, stroke, or death. In this article, the author highlights the potential for vascular wall imaging methods to detect and monitor vascular inflammation before occlusion occurs and reviews the evidence for effective therapy with biological agents in the treatment of this uncommon disease.
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• Takayasu arteritis is a serious vasculitis of the young, preferentially affecting women of Asian descent.
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• In Takayasu arteritis, a granulomatous panarteritis can lead to stenosis or occlusion of the aorta and its major branches, producing life-threatening ischemia of the arms or brain.
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• Early symptoms of Takayasu arteritis can be constitutional, potentially delaying diagnosis.
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• Neurologic complications of Takayasu arteritis include cerebral and spinal ischemia, typically secondary to hypoperfusion rather than embolic phenomena, but most commonly, patients report symptoms of presyncope/syncope, lightheadedness, dizziness, and headache during the course of the disease.
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• International diagnostic criteria are based on features of occlusive arterial disease, characteristic of Takayasu arteritis, but detection of early-stage disease when only nonspecific symptoms are present requires newer vessel wall imaging approaches, such as high resolution MRI or FDG-PET scanning.
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• Takayasu arteritis responds variably to traditional treatment with steroids and immunosuppressive agents; targeted therapies using monoclonal antibodies blocking actions of TNF-alpha and interleukin-6 have shown effectiveness producing improved relapse-free survival.
Historical note and terminology
In 1830 Rokushu Yamamoto published the first description of Takayasu disease in a case report from his private practice (64). In it, Yamamoto, who practiced Japanese oriental medicine, described a 45-year-old man who initially had a high fever and who presented a year later with no pulse in 1 arm, and a weak pulse in the other. Later, both carotid arteries became nonpalpable, and he suddenly died in the 11th year of follow-up.
Mikito Takayasu gave the first scientific presentation of this disease (64) in 1905 at the 12th Annual Meeting of the Japanese Ophthalmology Society. He presented a case of a 21-year-old woman with vision loss whose optic fundi exhibited a peculiar wreath-like anastomosis around the optic disc (88). At the same meeting in 1905, Onishi and Kagosha each contributed cases of their own, in which the same characteristic ocular findings were noted; in addition, both pointed out that no radial pulses could be palpated in their patients. In 1920, in the first necropsy case (of a 25-year-old woman), Otha confirmed that Takayasu disease is a panarteritis involving intima, media, and adventitia, and that the characteristic optic fundus finding resulted from ischemia due to obstruction of the carotid artery. In 1951 Shimizu and Sano summarized the clinical features of Takayasu disease in an English journal under the name of "pulseless disease." The 1992 Chapel Hill Consensus Conference defined Takayasu disease as granulomatous inflammation of the aorta and its main branches that usually occurs in female patients younger than 40 years old (33). According to the criteria of classification of the 1990 American College of Rheumatology, a case of vasculitis can be classified as Takayasu disease if 3 out of the following 6 criteria are fulfilled (sensitivity: 90.5%, specificity: 97.8%): (1) age at disease onset 40 years or younger, (2) claudication of the extremities, (3) decreased brachial artery pulse, (4) blood pressure difference between both arms of more than 10 mm Hg, (5) bruits over subclavian arteries or aorta, and (6) arteriographic abnormalities (03). It is important to note that these criteria were validated by comparison of Takayasu arteritis patients with control patients having other vasculitic disorders; it is evident that these criteria presume exclusion of those with identifiable alternative causes of vascular occlusion such as atherosclerosis.
There are challenges for detecting Takayasu arteritis in young children because the American College of Rheumatology criteria for diagnosis is designed for adults. A consensus to define realistic classification criteria for common childhood vasculitides was updated in 2010. According to the European League Against Rheumatism, Paediatric Rheumatology European Society, and the Paediatric Rheumatology International Trials Organisation consensus criteria, the diagnosis of Takayasu arteritis requires angiographic abnormalities (conventional CT, or MRI/A) of the aorta or 1 of its major branches and pulmonary arteries plus 1 or more of the following: (1) claudication or decreased peripheral artery pulses, (2) blood pressure difference greater than 10 mm Hg in any limb, (3) bruits of the aorta or its major branches, (4) hypertension, and (5) elevated acute phase reactant (68).