Takayasu arteritis
| Takayasu's arteritis | |
|---|---|
 |
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| Left anterior oblique angiographic image of Takayasu's arteritis showing areas of stenosis in multiple great vessels | |
| Classification and external resources | |
| Specialty | rheumatology |
| ICD-10 | M31.4 |
| ICD-9-CM | 446.7 |
| OMIM | 207600 |
| DiseasesDB | 12879 |
| MedlinePlus | 001250 |
| eMedicine | med/2232 ped/1956 neuro/361 radio/51 |
| MeSH | D013625 |
Takayasu's disease (also known as "aortic arch syndrome," "nonspecific aortoarteritis," and "pulseless disease") is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, most commonly affecting often young or middle-age women of Asian descent, though anyone can be affected. It mainly affects the aorta (the main blood vessel leaving the heart) and its branches, as well as the pulmonary arteries. Females are about 8–9 times more likely to be affected than males.
Those with the disease often notice symptoms 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 Takayasu's arteritis. Takayasu's arteritis is similar to other forms of vasculitis, including giant cell arteritis which typically affects older individuals. Due to obstruction of the main branches of the aorta, including the left common carotid artery, the brachiocephalic artery, and the left subclavian artery, Takayasu's arteritis can present as pulseless upper extremities (arms, hands, and wrists with weak or absent pulses on the physical examination) which may be why it is also commonly referred to as the "pulseless disease." Involvement of renal arteries may lead to a presentation of renovascular hypertension.
Some people develop an initial "inflammatory phase" characterized by systemic illness with signs and symptoms of malaise, fever, night sweats, weight loss, joint pain, fatigue, and fainting. Fainting may result from subclavian steal syndrome or carotid sinus hypersensitivity. There is also often anemia and marked elevation of the ESR or C-reactive protein (nonspecific markers of inflammation). The initial "inflammatory phase" is often followed by a secondary "pulseless phase". The "pulseless phase" is characterized by vascular insufficiency from intimal narrowing of the vessels manifesting as arm or leg claudication, renal artery stenosis causing hypertension, and neurological manifestations due to decreased blood flow to the brain.
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