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Key Features

  • Granulomatous vasculitis of the aorta and its major branches

  • Rare in North America but more prevalent in the Far East

  • Primarily affects women and typically has its onset in early adulthood

  • Chronic relapsing and remitting course that requires ongoing monitoring and adjustment of therapy

Clinical Findings

  • Can present with nonspecific constitutional symptoms

    • Malaise

    • Fever

    • Weight loss

    • Manifestations of vascular inflammation and damage

      • Diminished pulses

      • Unequal blood pressures in the arms

      • Carotidynia (tenderness over the carotid arteries)

      • Bruits over carotids and subclavian arteries

      • Retinopathy

      • Limb claudication

      • Hypertension

Diagnosis

  • No specific laboratory abnormalities

  • Erythrocyte sedimentation rate and the C-reactive protein level are elevated in most cases

  • 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

  • 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 the prednisone may be more effective than the prednisone alone

  • Biologic therapy with either infliximab tocilizumab may be effective for patients refractory to prednisone

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