Takayasu's arteritis

Takayasu's arteritis
Classification and external resources
File:Takayasu Arteritis.jpg
ICD-10	M31.4
ICD-9	446.7
OMIM	207600
DiseasesDB	12879
MedlinePlus	001250
eMedicine	med/2232 ped/1956 neuro/361 radio/51
MeSH	D013625

Takayasu's arteritis (also known as "Aortic arch syndrome", "Nonspecific Aortoarteritis" and "Pulseless disease") is an inflammatory disease with an unknown cause. It affects the aorta, the main blood vessel from the heart, as well as the blood vessels that attach to it. Although it has been reported worldwide, it is more common in young Asian women. Females are about 8-9 times more likely to get it than males. People usually get the disease between 15 and 30 years of age. In the Western world, atherosclerosis is a more frequent cause of obstruction of the aortic arch vessels than is Takayasu's arteritis.

It is also known as "Pulseless disease" because pulses on the upper extremities, such as the wrist pulse, may not be able to be felt.

History

The first case of Takayasu’s arteritis was described in 1908 by Dr. Mikito Takayasu at the Annual Meeting of the Japan Ophthalmology Society. Dr. Takayasu described a peculiar "wreathlike" appearance of blood vessels in the back of the eye (retina). Two Japanese colleagues at the same meeting (Dr. Onishi and Dr. Kagoshima) reported similar eye findings in patients whose wrist pulses were absent. It is now known that the blood vessel malformations that occur in the retina are an angiogenic response to arterial narrowings in the neck, and that the absence of pulses noted in some patients occur because of narrowings of blood vessels to the arms. The eye findings described by Takayasu are rarely seen in patients from North America and British Columbia.

Pathophysiology

Although its etiology is unknown, the condition is characterized by segmental and patchy granulomatous inflammation of the aorta and major derivative branches. This inflammation leads to arterial stenosis, thrombosis, and aneurysms.

Symptoms

About half of all patients develop an initial systemic illness with symptoms of malaise, fever, night sweats, weight loss, arthralgia, and fatigue. There is often an anemia and marked elevation of the ESR. This phase gradually subsides and is followed by a more chronic stage characterized by inflammatory and obliterative changes in the aorta and its branches. The other half of patients with Takayasu's arteritis present with only late vascular changes, without an antecedent systemic illness. In the late stage, weakness of the arterial walls may give rise to localized aneurysms. Raynaud's phenomenon is commonly found in this disease.

Treatments

The great majority of patients with Takayasu’s arteritis respond to prednisone. The usual starting dose is approximately 1 milligram per kilogram of body weight per day (for most people, this is approximately 60 milligrams a day). Because of the significant side effects of long-term high–dose prednisone use, the starting dose is tapered over several weeks to a dose that the physician feels is tolerable for the patient.

Surgical options may need to be explored for patients who do not respond to steroids. Re-perfusion of tissues can be achieved by large vessel reconstructive surgery such as bypass grafting. Grafting autologous tissue has the highest rates of success. Percutaneous transluminal coronary angioplasty (PTCA) are not as effective in the long term but has fewer risks.

External links

• Johns Hopkins Vasculitis Center Discusses Takayasu's Arteritis
• Takayasu's Arteritis Research Association
• 00389 at CHORUS
• -362086386 at GPnotebook

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