| Criterion | Definition |
| 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 |
| Bruits | Audible murmurs or palpable thrills over large arteries |
| Hypertension | 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) |