Takayasu arteritis

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Takayasu arteritis (aortic arch syndrome) is a systemic vasculitis of large vessels characterized by granulomatous inflammation of the aorta and its major branches. It most commonly affects Asian women < 40 years of age. Patients typically present with constitutional symptoms, decreased bilateral brachial and radial pulses (the disease is also known as pulseless disease), angina, and syncope. Imaging (e.g., MR angiography) is used to confirm the diagnosis; findings include vascular wall thickening and contrast enhancement, and luminal stenosis or occlusion. Biopsy of the affected vessels can also confirm the diagnosis but is not routinely indicated. Management typically involves immunosuppressive agents (e.g., high-dose glucocorticoids plus methotrexate); surgical therapy (e.g., vascular bypass) is indicated for patients with limb ischemia.

Granulomatous inflammation of the aorta and its major branches, resulting in thickening and stenosis of the involved blood vessels and subsequent vascular symptoms ^[2]

• Peak incidence: 15–45 years of age
• Most commonly affects individuals of Asian heritage
• ♀ > ♂ (9:1) ^[3]

Epidemiological data refers to the US, unless otherwise specified.

• Nonspecific symptoms: Fever, malaise, arthralgia, night sweats
• Vascular symptoms
  • Decreased bilateral brachial and radial pulses (so-called pulseless disease)
  • Syncope, angina pectoris
  • Bilateral carotid bruits
  • Impaired vision
  • Movement-induced muscular pain in one or more limbs
  • Raynaud phenomenon
  • Hypertension
• Skin manifestations
  • Erythema nodosum
  • Urticaria

You can't TAKe a pulse in TAKayasu arteritis (pulseless disease).

General principles ^[4]

• Diagnosis is primarily clinical but always requires confirmation with imaging or biopsy.
• Nonspecific laboratory findings may support the diagnosis: e.g., ↑ ESR, ↑ CRP, leukocytosis, anemia ^[5][6]
• Serial measurements of CBC and inflammatory markers may be used for follow-up.

Imaging studies ^[5][7][8]

Imaging of the aorta and major branches involves:

• MR angiography (MRA)
  • Indications: preferred initial study for all patients
  • Findings
    • Thickening and contrast enhancement of the vascular wall (i.e., inflammation)
    • Luminal stenosis or occlusion
• Alternatively
  • CT angiography (CTA)
    • More readily available and has higher resolution than MRA
    • Findings: similar to MRA
  • Angiography ; ^[6][7]
    • Consider for patients with planned vascular interventions (e.g., angioplasty, stenting).
    • Detects vascular stenosis
• Echocardiography: Consider for all patients to assess for aortic regurgitation and ventricular hypertrophy.

Diagnostic confirmation frequently relies on imaging, as biopsy is not always possible because affected vessels are often inaccessible. ^[9]

Biopsy of affected vessel ^[4][6][8]

• Indications
  • Not routinely required
  • Consider for patients with planned vascular interventions.
• Supportive findings
  • Granulomatous thickening of the aortic arch
  • Plasma cells and lymphocytes in the media and adventitia
  • Vascular fibrosis

General principles ^[5][9]

• Consult rheumatology for all patients.
• Pharmacotherapy with immunosuppressants is the mainstay of treatment.
  • Regimens are guided by disease severity and activity.
  • Goal is to achieve and maintain clinical remission
• Surgical therapy is reserved for selected cases.

Pharmacotherapy ^[5][9]

• Initial therapy: Start remission induction therapy for patients with active disease.
  • High-dose glucocorticoids
  • PLUS a glucocorticoid-sparing agent, e.g.: ^[5][9]
    • Methotrexate
    • Azathioprine
    • Cyclophosphamide ^[5]
    • TNF inhibitor (e.g., infliximab)
• Subsequent therapy: based on response to initial therapy
  • Clinical remission achieved
    • Taper and discontinue glucocorticoids.
    • Continue glucocorticoid-sparing agent.
  • Clinical remission not achieved (relapsed or refractory disease)
    • Continue glucocorticoids.
    • Consider changing to another glucocorticoid-sparing agent.
    • Consider referral for surgical therapy.

Glucocorticoid monotherapy is associated with a high relapse rate (∼ 70%). ^[5]

Surgical therapy ^[5][6][9]

• Indications
  • Elective procedures: progressive ischemia despite escalation of immunosuppressive agents
  • Emergency procedures
    • Critical stenosis of the aortic arch or its branches
    • Symptomatic neurological complications (e.g., cranial and/or cervical vessel stenosis)
    • Aneurysm dissection
• Surgical interventions include:
  • Vascular bypass
  • Vascular graft
  • Percutaneous transluminal angioplasty
  • Stent placement

Except for emergency indications, surgical intervention should be delayed until the patient is stable to decrease the risk of complications and achieve higher patency rates. ^[5]

Long-term monitoring

• Clinical evaluation (e.g., every 3–6 months )
• Regular imaging with noninvasive studies (e.g., MRA, CTA)
• Consider monitoring with inflammatory markers.

Supportive care

• Educate all patients on ASCVD prevention. ^[5]
• Prevent complications of glucocorticoid therapy.
• Monitor for adverse effects of immunosuppressants.
• Consider pneumocystis pneumonia prophylaxis.