Takayasu arteritis is a granulomatous vasculitis of the aorta and its major branches.

Rare in North America but more prevalent in the Far East, it primarily affects women and typically has its onset in early adulthood.

Clinical Findings

Takayasu arteritis can present with nonspecific constitutional symptoms of malaise, fever, and weight loss or with manifestations of vascular inflammation and damage: diminished pulses, unequal blood pressures in the arms, carotidynia (tenderness over the carotid arteries), bruits over carotid and subclavian arteries, retinopathy, limb claudication, and hypertension.

There are no specific laboratory abnormalities; the ESR and the CRP level are elevated in most cases.

The diagnosis is established by imaging studies, usually MRI, which can detect inflammatory thickening of the walls of affected vessels, or CT angiography, which can provide images of the stenoses, occlusions, and dilations characteristic of arteritis.

Treatment and Management

High-dose corticosteroids (e.g., oral prednisone, 1 mg/kg) are recommended for newly diagnosed or severely relapsing Takayasu arteritis.

The addition of methotrexate, azathioprine, mycophenolate mofetil, or TNF inhibitors is recommended for refractory disease and as steroid-sparing agents; TNF inhibitors are first-line additions.

There are limited data for other biologic therapies, such as anti-IL6 therapy (tocilizumab) and tofacitinib.

Takayasu arteritis has a chronic relapsing and remitting course that requires ongoing monitoring and adjustment of therapy.

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