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  • Updated 03.23.2023
  • Released 08.13.2001
  • Expires For CME 03.23.2026

Takayasu arteritis



Takayasu arteritis is a primary systemic large-vessel vasculitis that affects the aorta and its major branches by producing mainly stenotic and occlusive lesions. Takayasu arteritis typically occurs in young women. Constitutional and musculoskeletal symptoms are frequent but nonspecific. Vascular manifestations include extremity claudication and cardiac failure. Up to 20% of patients develop cerebrovascular events, such as transient ischemic attack or stroke. This review provides an extensive overview of epidemiological, clinical, laboratory, and vascular imaging and therapeutic aspects of Takayasu arteritis. Methods of monitoring disease activity and vascular progression, as well as advances in pharmacological therapies and interventional procedures, have been updated.

Key points

• Takayasu arteritis is a primary systemic large-vessel vasculitis affecting the aorta and its major branches and is characterized by a stenosing and occlusive nature and relapsing course.

• Takayasu arteritis usually affects young females with a heterogeneous presentation, usually with constitutional and musculoskeletal symptoms besides ischemic manifestations affecting the extremities, hypertension, and other features derived from vascular inflammatory involvement.

• Although catheter-directed angiography is the gold standard for studying the arterial lumen more precisely, CT angiography and MR imaging and angiography (MRI and MRA) can be used for the initial Takayasu arteritis diagnosis. MRI and MRA are preferred for sequential vascular imaging during follow-up.

• Normal acute phase reactants can be misleading in Takayasu arteritis due to the lack of correlation with progression of vascular stenoses. Disease activity is still assessed by the combination of clinical manifestations, elevated erythrocyte sedimentation rate (ESR), and changes in vascular imaging techniques.

• Glucocorticoids are the treatment of choice to induce and maintain remission, but additional immunosuppressive agents, preferably methotrexate, are frequently needed. In patients with Takayasu arteritis refractory to these first-line agents, tocilizumab and anti-TNF agents have been demonstrated to be good therapeutic options. However, the duration of their administration is still unclear.

• Among revascularization procedures, bypass with autologous vessel grafts have shown better long-term patency than other procedures. The use of angioplasty has also been reported to have better long-term results than artery stenting. If possible, revascularization should be performed when the disease is in maintained remission.

• Diagnosis and control of hypertension are crucial because hypertension is frequently unrecognized due to stenoses of large vessels that provide blood supply to the extremities.

Historical note and terminology

Definition, historical perspective, and classification criteria. Takayasu arteritis is a primary large-vessel vasculitis with a predilection for the aorta and its primary branches. Chronic inflammatory lesions often lead to stenotic or occlusive lesions and less frequently evolve to dilation and aneurysm formation (50).

The disease takes the name of Dr. Mikito Takayasu, an ophthalmologist who presented a young girl with retinal changes of chronic hypoperfusion at a Japanese ophthalmology meeting in 1905. At the same meeting, Dr. Onishi and Dr. Kagoshima presented a patient with similar retinal changes and the absence of radial pulses (95). Previously, the first description of Takayasu arteritis in the literature was by Rokushu Yamamoto in 1830 (95). A 45-year-old man presented with fever; a year later, he developed abnormal radial pulses in both arms and a subsequent absence of pulse in both carotid arteries. The patient died suddenly 11 years later.

Different classification criteria have been used for Takayasu arteritis in the last few decades. In the 1994 (46) and 2013 (47) Chapel Hill Consensus Conference on the Nomenclature of Vasculitides, Takayasu arteritis was defined as a large-vessel arteritis, often granulomatous and predominantly affecting the aorta and its major branches. Disease onset was usually in patients younger than 50 years of age. The 1990 American College of Rheumatology criteria for the classification of Takayasu arteritis proposed the next criteria: (1) age at disease onset of 40 years or younger; (2) claudication of the extremities; (3) decreased brachial artery pulse; (4) blood pressure difference between both arms of more than 10 mmHg; (5) bruits over subclavian arteries or aorta; and (6) arteriographic abnormalities. A patient could be classified with Takayasu arteritis if three of the six criteria were fulfilled, with a sensitivity of 90.5% and a specificity of 97.8% (02).

The most recent 2022 ACR/European League Against Rheumatism (EULAR) classification criteria for Takayasu arteritis include the identification of medium- or large-vessel vasculitis and the exclusion of other potential causes or mimickers (29). The final 12 criteria are described in Table 1.

Table 1. Diagnostic Criteria for Takayasu Arteritis

Absolute requirements

• Age ≤ 60 years at diagnosis

• Evidence of vasculitis on imaging

Additional clinical criteria

• Female sex


• Angina or ischemic cardiac pain


• Arm or leg claudication


• Vascular bruit


• Reduced pulse by physical examination in arms


• Reduced pulse or tenderness of carotid arteries


• Systolic blood pressure difference in arms ≥ 20 mm Hg


Additional imaging criteria

• Number of affected arterial territories (select one)

One arterial territory


Two arterial territories


> Two arterial territories


• Symmetric involvement of paired arteries


• Abdominal aorta (renal or mesenteric) involvement


≥ 5 points is needed for the diagnosis

Slightly modified from (29)

The EULAR, the Paediatric Rheumatology European Society (PRES), and the Paediatric Rheumatology International Trials Organisation (PRINTO) updated consensus classification criteria for common childhood vasculitides in 2010 (99). The classification of children with Takayasu arteritis requires typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of the following five criteria: (1) decreased peripheral pulse or claudication; (2) blood pressure discrepancy higher than 10 mmHg in any limb; (3) bruits on the aorta or its major branches; (4) hypertension; and (5) elevated acute phase reactants.

The previous angiographic Takayasu arteritis classifications were updated by Hata and colleagues at the Takayasu Conference in 1994 (35). Vascular topographic extent was divided into four types (Table 2). In addition, the involvement of coronary or pulmonary arteries should be indicated as C (+) or P (+), respectively.

Table 2. Angiographic Takayasu Arteritis Classification




Involvement of primary branches of the aortic arch


Involvement of the ascending aorta, aortic arch, and its branches


Involvement of the ascending aorta, aortic arch and its branches, and thoracic descending aorta


Involvement of the thoracic descending aorta, abdominal aorta, and/or renal arteries


Involvement of the abdominal aorta and/or renal arteries


A combination of features of both types lIb and IV

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