Takayasu arteritis

Last updated: December 8, 2022

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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 [1]

  • Peak incidence: 15–45 years of age
  • Most commonly affects individuals of Asian heritage
  • > (9:1) [2]

Epidemiological data refers to the US, unless otherwise specified.

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

General principles [3]

  • 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 [4][5]
  • Serial measurements of CBC and inflammatory markers may be used for follow-up.

Imaging studies [4][6][7]

Imaging of the aorta and major branches involves:

Diagnostic confirmation frequently relies on imaging, as biopsy is not always possible because affected vessels are often inaccessible. [8]

Biopsy of affected vessel [3][5][7]

  • Indications
    • Not routinely required
    • Consider for patients with planned vascular interventions.
  • Supportive findings

General principles [4][8]

  • 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 [4][8]

Glucocorticoid monotherapy is associated with a high relapse rate (∼ 70%). [4]

Surgical therapy [4][5][8]

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. [4]

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

  1. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  2. Onen F, Akkoc N. Epidemiology of Takayasu arteritis. Presse Med. 2017; 46 (7-8): p.e197-e203. doi: 10.1016/j.lpm.2017.05.034 . | Open in Read by QxMD
  3. Kim ESH, Beckman J. Takayasu arteritis: challenges in diagnosis and management.. Heart. 2018; 104 (7): p.558-565. doi: 10.1136/heartjnl-2016-310848 . | Open in Read by QxMD
  4. Maz M, Chung SA, Abril A, et al. American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Care Res. 2021; 73 (8): p.1071-1087. doi: 10.1002/acr.24632 . | Open in Read by QxMD
  5. Seyahi E. Takayasu arteritis: an update. Curr Opin Rheumatol. 2017; 29 (1): p.51-56. doi: 10.1097/bor.0000000000000343 . | Open in Read by QxMD
  6. Bardi M, Diamantopoulos AP. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice summary. Radiol Med (Torino). 2019; 124 (10): p.965-972. doi: 10.1007/s11547-019-01058-0 . | Open in Read by QxMD
  7. Espígol-Frigolé G, Prieto-González S, Alba MA, et al. Advances in the Diagnosis of Large Vessel Vasculitis. Rheumatic Disease Clinics of North America. 2015; 41 (1): p.125-140. doi: 10.1016/j.rdc.2014.10.001 . | Open in Read by QxMD
  8. Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2019; 79 (1): p.19-30. doi: 10.1136/annrheumdis-2019-215672 . | Open in Read by QxMD

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