Takayasu Arteritis

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) is recommended for newly diagnosed or severely relapsing disease

• The addition of TNF inhibitors (first-line additions), methotrexate, azathioprine, or mycophenolate mofetil is recommended for refractory disease and as corticosteroid-sparing agents

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

• Chronic relapsing and remitting courses require ongoing monitoring and adjustment of therapy