Takayasu arteritis commonly occurs in woman younger than age 50 years; however, it has been reported in patients as young as age 6 months ( Pediatric Takayasu Arteritis). Takayasu arteritis can manifest as isolated, atypical, and/or catastrophic disease. It can involve any or all of the major organ systems. The disease has been reported in all parts of the world, although it appears to be more prevalent in Asians. (See Epidemiology.)

Signs and symptoms

Approximately 10% of patients with Takayasu arteritis are asymptomatic. Clinical manifestations are heterogeneous and include the following:

Systemic (eg, malaise, arthralgias, fever) – May precede clinical vascular involvement
Cardiac and vascular (eg, bruit, especially carotid; blood pressure difference of extremities, claudication, hypertension)
Neurologic (eg, headache, visual disturbance)
Dermatologic (eg, erythema nodosum)

Diagnosis

The American College of Rheumatology classification for Takayasu arteritis requires the presence of 3 of the following 6 criteria:

Age of 40 years or younger at disease onset
Claudication of the extremities
Decreased pulsation of one or both brachial arteries
Difference of at least 10 mm Hg in systolic blood pressure between arms
Bruit over one or both subclavian arteries or the abdominal aorta
Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the upper or lower extremities that is not due to arteriosclerosis, fibromuscular dysplasia, or other causes

Takayasu arteritis can be divided into the following six types based on angiographic involvement:

Type I – Branches of the aortic arch
Type IIa – Ascending aorta, aortic arch, and its branches
Type IIb – Type IIa region plus thoracic descending aorta
Type III – Thoracic descending aorta, abdominal aorta, renal arteries, or a combination
Type IV – Abdominal aorta, renal arteries, or both
Type V – Entire aorta and its branches

Treatment

Corticosteroids are the mainstay of therapy for active Takayasu arteritis. However, some patients may also require cytotoxic agents, to achieve remission and taper of long-term corticosteroid treatment. Biologic agents (eg, tocilizumab, tumor necrosis factor inhibitors) have shown encouraging results.