Criteria of Ishikawa for the Diagnosis of Takayasu Arteritis

Obligatory criteria:

(1) age < 40 years: at diagnosis OR at onset of "characteristic signs and symptoms" of 1 month duration in patient history. Characteristic signs and symptoms include: limb claudication, pulselessness or pulse difference, unobtainable blood pressure or a significant blood pressure difference > 10 mm in systolic pressure, fever, neck pain, transient amaurosis, blurred vision, syncope, dyspnea or palpitations.

Major criteria:

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

(2) right mid-subclavian artery lesion: similar to left subclavian artery lesion

Minor criteria:

(1) high erythrocyte sedimentation rate (ESR): Unexplained, persistent high ESR > 20 mm/h (Westergren method) at diagnosis, or recorded in patient history.

(2) carotid artery tenderness: Unilateral or bilateral tenderness of common carotid arteries by physician palpation. Neck muscle tenderness is unacceptable.

(3) hypertension: Persistent blood pressure > 140/90 mm Hg brachial or > 160/90 mm Hg popliteal at age < 40 years. Or recorded in medical history at age < 40 years.

(4) aortic regurgitation or annuloaortic ectasia: Demonstrated by Doppler echocardiography or angiography; regurgitation also by auscultation.

(5) pulmonary artery lesion: (a) Lobar or segmental arterial occlusion or equivalent determined by angiography or perfusion scintigraphy; or (b) 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 artery lesion: The 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: The presence of the most severe stenosis or occlusion in the distal third lesion determined by angiography.

(8) descending thoracic aorta lesion: Narrowing, dilatation, or aneurysm, luminal irregularity or any lesion combination determined by angiography. Tortuosity alone is not acceptable.

(9) abdominal aorta lesion: Narrowing, dilatation or aneurysm, luminal irregularity or any combination  determined by angiography; AND absence of lesion in aorto-iliac region consisting of the distal 2 cm of terminal aorta and bilateral common iliac arteries. Tortuosity alone is not acceptable.

High probability for the diagnosis of Takayasu arteritis - one or more of the following:

 (1) obligatory AND 2 major criteria

(2) obligatory AND 1 major AND >= 2 minor criteria

(2) obligatory AND >= 4 minor criteria

Performance:

• The sensitivity in the original series of 96 patients was 84%.

• There is some geographical and racial variation in the disease. The criteria may underdiagnose the disease in some parts of the world since all of the patients in the series were Japanese.

• The age requirement does not allow diagnosis of patients with late onset disease.

• Sharma et al list features of the criteria that they felt limited their overall use in diagnosis.