Final EULAR/PRINTO/PRES c-TA criteria (with glossary) and classification definition (sample 1056)
| Criterion | Glossary | Sensitivity (%) | Specificity (%) | AUC (%) |
|---|---|---|---|---|
| Angiographic abnormality (mandatory criterion) | Angiography (conventional, CT, or MRI) 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 | 100 | 99.9 | 99.9 |
| 1. Pulse deficit or claudication | Lost/decreased/unequal peripheral artery pulse(s) | 74.7 | 99.1 | 86.9 |
| Claudication: focal muscle pain induced by physical activity | ||||
| 2. Blood pressure (BP) discrepancy | Discrepancy of four limb systolic BP >10 mm Hg difference in any limb. | 63.5 | 99.6 | 81.6 |
| 4. Bruits | Audible murmurs or palpable thrills over large arteries | 58.8 | 99.8 | 79.3 |
| 5. Hypertension | Systolic/diastolic BP greater than 95th centile for height | 63.2 | 90.5 | 76.8 |
| 6. Acute phase reactant | Erythrocite sedimentation rate >20 mm per first hour or CRP any value above normal (according to the local laboratory) | 95.0 | 14.1 | 54.6 |
| c-TA EULAR/PRINTO/PRES Ankara 2008 classification definition: κ 0.99 (95% CI 0.93 to 1.00) | Angiographic abnormalities of the aorta or its main branches and pulmonary arteries showing aneurysm/dilatation (mandatory criterion) plus one of the five following criteria: | 100 | 99.9 | 99.9 |
| Pulse deficit or claudication | ||||
| Four limbs BP discrepancy | ||||
| Bruits | ||||
| Hypertension | ||||
| Acute phase reactant |
AUC, area under the curve; CRP, C-reactive protein; c-TA, c-Takayasu arteritis; EULAR, European League Against Rheumatism; PRES, Paediatric Rheumatology European Society; PRINTO, Paediatric Rheumatology International Trials Organisation.