Takayasu's arteritis also known as, "aortic arch syndrome," "nonspecific

aortoarteritis," and "pulseless disease”, is a form of large granulomatous

vasculitis with massive intimal fibrosis and vascular narrowing, mainly
affecting aorta and its branches, as well as the pulmonary arteries.
The first case of Takayasu’s arteritis was described in 1908 by
Japanese ophthalmologist Mikito Takayasu at the Annual Meeting of the
Japan Ophthalmology Society. Takayasu described a peculiar "wreathlike"
appearance of the blood vessels in the back of the eye (retina).
Two Japanese physicians at the same meeting (Drs. Onishi and
Kagoshima) reported similar eye findings in individuals whose wrist
pulses were absent.
It most commonly affects young or middle-age women of Asian descent.
Females are about 8–9 times more likely to be affected than males.
Worldwide incidence of Takayasu arteritis is estimated at 2.6 cases per
What is the global prevalence of
million per year.

Takayasu arteritis?
Updated: Nov 14, 2018

 Author: Jefferson R Roberts, MD; Chief Editor: Herbert S Diamond, MD more...

 References
The incidences of TA were estimated to be 1–2 per million in Japan and
2.2 per million in Kuwait. Recent epidemiologic studies suggest that TA is
being increasingly recognized in Europe with reported incidence estimates
varying from 0.4 to 1.5 per million. The highest ever prevalence of TA at 40
per million was estimated in Japan and the lowest ever one at 0.9 per
million in US. 1
The disease commonly presents between the ages of 10 and 20 years, with three-quarters of
patients presenting in this period, with a ratio of males to females of 1 to 8.5.5 There is,
nonetheless, a wide range of presenting age, with some presenting as early as 24 months.6 A
delay between symptoms and diagnosis of 2 to 11 years is seen in the west,5 with greater delay
observed in juveniles as opposed to adult populations.7 A study in India8 of patients with
Takayasu’s disease under 18 years of age reported a delay of only 2.5 to 5.5 months. This
presumably reflects the higher incidence in the Indian subcontinent, and thus increased clinical
awareness. Takayasus disease: A review Article in Cardiology in the Young · July 2008
The cellular and biochemical processes involved in the pathogenesis of
TAK are beginning to be elucidated, and implicate both cell and antibody-
mediated autoimmune disease supported by the genetic association with
HLA-B∗52. Those with the disease often notice symptoms between 15 and
30 years of age.
Clinical features include an initial "inflammatory phase" characterized by
malaise, fever, night sweats, weight loss, joint pain, fatigue is often followed
by a secondary "pulseless phase” 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.

Epidemiology of Takayasu arteritis

Author links open overlay panelFatosOnenNurullahAkkoc

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https://doi.org/10.1016/j.lpm.2017.05.034

The genetics of Takayasu arteritis

Author links open overlay panelPaulRenauer1Amr H.Sawalha12

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https://doi.org/10.1016/j.lpm.2016.11.031