Angiographic abnormality (mandatory criterion)

Angiography (conventional, computed tomography, or magnetic resonance imaging) of the aorta or its main branches and pulmonary arteries showing aneurysm/dilatation, narrowing, occlusion or thickened arterial wall not due to fibromuscular dysplasia, or similar causes; changes usually focal or segmental

Pulse deficit or claudication

Lost/decreased/unequal peripheral artery pulse(s)

Claudication: focal muscle pain induced by physical activity

Blood pressure discrepancy

Discrepancy of four limb systolic blood pressure > 10 mmHg difference in any limb


Audible murmurs or palpable thrills over large arteries


Systolic/diastolic blood pressure greater than 95th percentile for height

Acute phase reactants

Erythrocyte sedimentation rate > 20 mm per first hour or C-reactive protein any value above normal (according to the local laboratory)