Takayasu arteritis, a form of granulomatous arteritis, which affects
large- and medium-sized arteries, primarily the aorta and its large
branches as well as proximal portions of pulmonary, coronary, and
renal arteries, is also known as "Pulseless disease" and "Occlusive
Rokushu Yamamoto, who practiced medicine in Japan, published the first
description of Takayasu arteritis in 1830.
first scientific presentation was given in 1905 by Mikito Takayasu,
Professor of ophthalmology in Japan.
disease is rare, with 5000 cases reported throughout Japan between
1990 and 2000, while a US study put the incidence at 2.6 cases per
1,000,000 inhabitants per year. The incidence in European countries is
condition is most commonly seen in Japan, South East Asia, India, and
Mexico. Although early reports suggested that the disease was confined
to females from Eastern Asia, it is now recognized worldwide and
affects both sexes. Disease manifestations vary between regions, and
women are affected in 80–90% of cases in the majority cases.
disease commonly presents in the second or third decade of life, with
a delay in diagnosis of between months and years from the onset of
first symptoms. However, the illness may also present in childhood.
the early phase patients may complain of systemic symptoms including
fever, weight loss, malaise, myalgia, carotidynia, and headaches.
These symptoms are often insidious and frequently missed, or their
significance is overlooked.
later pulseless phase, commonly appearing months or years later,
reflects end-organ ischemia . Focal manifestations differ according to
the anatomical location of the affected arteries. Symptoms of upper
and lower limbs claudication, light headedness, and chest pain
predominate. Decreased or absent peripheral pulses, aortic
regurgitation, arterial bruits, and both systemic and pulmonary
hypertension are common.
Histologically, initially, there are mononuclear cell infiltrations in
the adventitia and granulomas with Langerhans cells in the media,
followed by disruption of the elastin layer and subsequent massive
medial and intimal fibrosis.
These lesions result
in segmental stenosis, occlusion, dilatation, and aneurysmal formation
in the affected vessels.
defined the four most important complications of Takayasu arteritis as
retinopathy, secondary hypertension, aortic regurgitation, and
aneurysm formation, each graded as mild, moderate, or severe.