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At a Glance

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An inflammatory chronic progressive panendarteritis of the aorta that occludes one or more of the large branches of the aortic arch, leading to hypoperfusion of major peripheral organs, including the brain and the spinal cord. Also known as the “pulseless women disease.” Symptoms consist of aphasia, transient hemiparesis, unilateral transient amblyopia or persistent blindness, headache, vertigo, and syncopal attacks. Progressive evolution with poor prognosis. Autoimmune in origin. Affects mostly young women.

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Synonyms

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Martorell Syndrome; Martorell-Fabré Syndrome; Reader-Harbitz Syndrome; Takayasu Arteritis; Takayasu-Martorell-Fabré Syndrome; Takayasu-Onishi Syndrome; Aortic Arch Arteritis; Maladie des Femmes sans Pouls (French appellation).

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History

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First described by Mikito Takayasu, a Japanese ophthalmologist, in 1908.

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Incidence

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1.2 to 2.5:1,000,000 population per year in whites; higher in Asian populations. Affects young women less than 30 years in 80 to 90% of cases. The female-to-male ratio is 8:1. In the United States, it is suggested that Takayasu arteritis affects approximately 1 person per 1000 in the general population. Internationally it afflicts 6 person per 1000. The mortality is usually caused by vascular complications such as hypertension, stroke and aortic insufficiency.

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Genetic Inheritance

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None; probably autoimmune.

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Pathophysiology

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Histology demonstrates granulomatous infiltration of the adventitia and media of arteries with lymphocytes, plasma cells, histiocytes, and giant cells. Following the acute phase of inflammation, a chronic sclerotic phase develops. Immune complexes have been demonstrated; however, an immune complex-mediated etiology has never been confirmed. Antiendothelial antibodies have been demonstrated by a number of workers, but elevated levels are not specific to Takayasu arteritis.

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Diagnosis

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Age of onset, clinical history, and examination. The diagnosis is confirmed by arteriography to assess degree of vascular involvement. Four types of late-phase are described:

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  • Type I is the classic pulseless form that involves the brachiocephalic vascular region, the carotid arteries, and the subclavian arteries.
  • Type II is a combination of type I and III.
  • Type III is represented by an atypical coarctation of the thoracic and abdominal portion of the aorta, distal to the arch and major branches.
  • Type IV affects the aorta in its entire length, causing an extensive dilatation. The major arterial branches of the aorta are also affected.

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The most common type is in 65% of patients, type III. The most common vessel involved is the subclavian artery (50%).

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Clinical Aspects

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Onset occurs at younger than 40 years of age. Clinical findings include limb claudication, multiple vascular bruits (carotid and aortic in 70% of patients), and less frequently the femoral and subclavian artery manifestations. Asymmetric pulse between limbs; vertebrobasilar insufficiency secondary to vessel stenosis; arthralgia and myalgia. Up to 30% of patients report visual disturbance, including amaurosis and diplopia. ...

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