Published descriptions of this form of arteritis date back to 1830. However, the disease received its name from Mikito Takayasu, a professor of ophthalmology, who in 1905 presented the case of a 21-year-old woman with characteristic fundal arteriovenous anastomoses, which later was suggested to result from retinal ischemia. At disease presentation or during relapses, TAK patients may present with nonspecific inflammatory complaints, such as fever, malaise, anorexia, weight loss, myalgia or arthralgias. This can be associated with vascular pain (e.g., carotidynia). As arterial lesions develop, features like decreased or absent peripheral pulses, vascular bruits, discrepancies in blood pressure between limbs, limb claudication, and hypertension arise. Heart failure can also be seen as a result of aortic regurgitation, longstanding hypertension, or coronary artery disease. Transient ischemic attacks, strokes, mesenteric ischemia, and retinopathy are other manifestations of the resultant ischemia. TAK can affect medium-size vessels, such as coronary arteries, and pulmonary vasculopathy and hypertension are also seen in some patients. Extravascular manifestations that have been reported include dermatologic manifestations, such as erythema nodosum and erythema induratum, glomerular lesions and cardiac manifestations, such as dilated cardiomyopathy and myocarditis.3
Takayasu’s arteritis is most commonly seen in Japan, South East Asia, India and Mexico. It is rare in North America, with an estimated incidence of 2.6 per million per year in Olmsted County, Minn.4 Although geographical differences regarding the distribution of arterial lesions have been described in TAK, the aorta is the most affected artery, followed by the subclavian, common carotid and renal arteries.5 The disease commonly presents in the second or third decade of life and predominantly affects women. From the onset of first symptoms, there is often a delay in diagnosis of months to years.
The American College of Rheumatology has established classification criteria for TAK (see Table 1), which were primarily designed to distinguish this disorder from other forms of vasculitis for study purposes. This classification system has a sensitivity and specificity of 90.5% and 97.8%, respectively.6 Attempts have been made to classify the disease on the basis of angiographic findings (see Figure 4), which can be useful in allowing a comparison of patient characteristics according to the involved vessels and helpful in planning surgery.7 However, this provides little information on prognosis.