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Criteria for the Classification of Takayasu Arteritis

Takayasu arteritis (TA) is a chronic large vessel vasculitis that affects aorta, its main branches and pulmonary arteries. The inflammatory process results in stenosis, occlusion, dilation or aneurysm formation in the arterial wall.

The 1990 American College of Rheumatology Classification Criteria for Takayasu arteritis

Criteria

Definition

Age at disease onset in year

Development of symptoms or findings related to Takayasu arteritis at age <40 years.

Claudication of extremities

Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities.

Decreased brachial artery pulse

Decreased pulsation of one or both brachial arteries

BP difference >10mmHg

Difference of >10mmHg in systolic blood pressure between arms

Bruit over subclavian arteries or aorta

Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta

Arteriogram abnormality

Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal uppper or lower extremities, not due arteriosclerosis, fibro-muscular dysplasia, or similar causes: changes usually focal or segmental

For purposes of classification, a patient shall be said to have Takayasu’s arteritis if at least three of these six criteria are present. The presence of any three or more criteria yields a sensitivity of 90.5% and a specificity of 97.8%. BP = blood pressure (systolic) difference between arms

 

Ishikawa’s Criteria for Takayasu’s Arteritis (Modified According to Sharma et al.)

Three major criteria:

  1. Left mid subclavian artery lesion: The most severe stenosis or occlusion present in the mid portion from the point 1 cm proximal to the vertebral artery orifice up to that 3 cm distal to the orifice determined by angiography

  2. Right mid subclavian artery lesion: The most severe stenosis or occlusion present in the mid portion from the right vertebral artery orifice to the point 3 cm distal to orifice determined by angiography

  3. Characteristic signs and symptoms of at least one month duration: These include limb claudication, pulselessness or pulse differences in limbs, an unobtainable  or significant blood pressure difference (> 10 mmHg systolic blood pressure difference in limb), fever, neck pain, transient amaurosis, blurred vision, syncope, dyspnea or palpitations.

Ten minor criteria

  1. High ESR: Unexplained persistent high erythrocyte sedimentation rate (ESR) >20 mm/h (Westergren) at diagnosis or presence of the evidence in patients history

  2. Carotid artery tenderness: Unilateral or bilateral tenderness of common arteries on palpation. Neck muscle tender ness is unacceptable

  3. Hypertension: Persistent blood pressure > 140/90 mmHg brachial or > 160/90 mmHg popliteal

  4. Aortic regurgitation or Anuloaortic ectasia: Aortic regurgitation by auscultation or Doppler echocardiography or angiography; or Anuloaortic ectasia  by angiography or two-dimensional echocardiography

  5. Pulmonary artery lesion: Lobar or segmental arterial occlusion or equivalent determined by angiography or perfusion scintigraphy, or presence of stenosis, aneurysm, luminal  irregularity or any combination in pulmonary trunk or in unilateral or bilateral pulmonary arteries determined by angiography.

  6. Left mid common carotid lesion: Presence of the most severe stenosis or occlusion in the mid portion of 5 cm in length from the point 2 cm distal to its orifice determined by angiography.

  7. Distal brachiocephalic trunk lesion:. Presence of the most stenosis or occlusion in the distal third determined by angiography

  8. Descending thoracic aorta lesion: Narrowing, dilatation or aneurysm, luminal irregularity or any combination determined by angiography: tortuosity alone is unacceptable.

  9. Abdominal aorta lesion: Narrowing, dilatation or aneurysm, luminal irregularity or aneurysm combination.

  10. Coronary artery lesion: Documented on angiography below the age of 30 years in the absence of risk factors like hyperlipidemia or diabetes mellitus

Presence of two major or one major and two minor criteria or four minor criteria suggests a high probability of Takayasu’s arteritis

 

References:

  1. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW Jr, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990 Aug;33(8):1129-34. [Medline]

  2. Sharma BK, Siveski-Iliskovic N, Singal PK. Takayasu arteritis may be underdiagnosed in North America. Can J Cardiol. 1995 Apr;11(4):311-6. [Medline]

  3. de Souza AW, de Carvalho JF. Diagnostic and classification criteria of Takayasu arteritis. J Autoimmun. 2014 Feb-Mar;48-49:79-83. [Medline]

 

Created: Dec 23, 2005
Update: Jan 02, 2021.

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