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
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).
First described by Mikito Takayasu, a Japanese
ophthalmologist, in 1908.
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
None; probably autoimmune.
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.
Age of onset, clinical history, and examination. The
diagnosis is confirmed by arteriography to assess degree of vascular
Four types of late-phase are described:
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.
The most common type is in 65% of patients, type III. The most common vessel
involved is the subclavian artery (50%).
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. Funduscopy may reveal microaneurysms, venous
beading, and in the later stages of the disease, hemorrhagic arteriovenous
anastomoses, optic atrophy, or retinal detachment. Cardiovascular findings
include main coronary artery stenoses, aortic regurgitation, cardiac
failure, aneurysm formation, arterial stenosis, and hypertension. Although
not always clinically evident, ...