This page has moved. Click here to view.


Takayasu's Arteritis

Takayasu's arteritis is a chronic inflammatory disease of  the aorta and its major branches. Takayasu's arteritis occurs worldwide, although the large majority of cases are seen in Asia and Africa. The incidence in North American and European populations is 1.2 to 2.6 per million per year. A specific cause has not been found, although the bulk of evidence favors an autoimmune etiology. It has been linked to rheumatic fever, streptococcal infections, rheumatoid arthritis, and other collagen vascular diseases. An association between the disease and certain HLA subtypes has been reported, although it is of unclear significance Takayasu's Arteritis, Takasu's arteritis.

In the early stage of the disease there is active inflammation involving a granulomatous arteritis of the aorta and its branches, with secondary alterations in the media and adventitia. The disease progresses at variable rates to a later sclerotic stage in which there is intimal hyperplasia, medial degeneration, and

Takayasu's arteritis most often involves the aortic arch and its major branches, with changes that are usually most marked at branch points in the aorta. It may present as multisegmental aortic disease with areas of normal wall between affected sites, as diffuse involvement of the aorta, or as disease of individual arteries arising from the aorta. The pulmonary artery may also be involved. Lesions are purely stenotic in 85 per cent of patients, purely dilatative in 2 per cent, and mixed in


The disease affects women much more frequently than men, in a ratio of 8:1. The mean age at the time of diagnosis is 29 years. In as many as three-fourths of cases, onset is in the teenage years, although cases beginning from infancy to late middle-age have been reported. Because the symptoms of Takayasu's arteritis are generally nonspecific, there may be a delay of months to years between the first appearance of symptoms and the time of diagnosis. In fact, only 6 per cent of the patients in 

At the time of diagnosis, 85 to 96 per cent of patients have entered the sclerotic phase of the disease and have symptoms of vascular insufficiency of either the upper extremities or, less commonly, the lower extremities. Patients with types I and III exhibit those findings most typical of the disease, namely "reversed" coarctation of the aorta with absent or diminished upper body pulses and 

Hypertension complicates this disease in 50 to 60 per cent of cases but may be difficult to recognize because of diminished pulses in the arms. Hypertension typically arises from renal artery stenosis and


The natural history of this uncommon disease has been best defined in a recent series by Ishikawa and Maetani, who followed 120 patients with Takayasu's arteritis for up to 15 years. The overall 15-year


Laboratory abnormalities during the acute systemic phase include an elevated sedimentation rate, a low-grade leukocytosis, and mild anemia of chronic disease. These return toward normal when the


Glucocorticoids in high doses (prednisone, 1 mg/kg body weight per day) are well established as the primary therapy of Takayasu's arteritis and often dramatically improve the constitutional symptoms, halt disease progression in patients in the systemic inflammatory stage, and 

In fact, the sedimentation rate, usually an accurate indicator of systemic disease activity, is quite useful in directing therapy. When patients fail to respond to steroid therapy, cyclophosphamide (2 mg/kg/day) has

Alternatively, low-dose methotrexate (about 0.3 mg/kg/week) may enhance the

Although medical therapy has been successful in improving symptoms in a majority of patients, it is not

The indications for surgery in the treatment of Takayasu's arteritis are 

One promising advance in the treatment of the obstructive lesions of Takayasu's arteritis is the use of percutaneous transluminal angioplasty. Tyagi et al. performed angioplasty for stenotic lesions of the