20-18: Takayasu Arteritis

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. 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. Corticosteroids (eg, oral prednisone, 1 mg/kg for 1 month, followed by a taper over several months to 10 mg daily) are the mainstays of treatment. The addition of methotrexate, azathioprine, or mycophenolate mofetil to prednisone may be more effective than the prednisone alone. Case series suggest that biologic therapy with either inhibitors of TNF (eg, infliximab) or the IL-6 receptor (tocilizumab) may be effective for patients refractory to prednisone. Takayasu arteritis has a chronic relapsing and remitting course that requires ongoing monitoring and adjustment of therapy.