Table 4

Final EULAR/PRINTO/PRES c-TA criteria (with glossary) and classification definition (sample 1056)

CriterionGlossarySensitivity (%)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 segmental10099.999.9
 1. Pulse deficit or claudicationLost/decreased/unequal peripheral artery pulse(s)74.799.186.9
Claudication: focal muscle pain induced by physical activity
 2. Blood pressure (BP) discrepancyDiscrepancy of four limb systolic BP >10 mm Hg difference in any limb.63.599.681.6
 4. BruitsAudible murmurs or palpable thrills over large arteries58.899.879.3
 5. HypertensionSystolic/diastolic BP greater than 95th centile for height63.290.576.8
 6. Acute phase reactantErythrocite sedimentation rate >20 mm per first hour or CRP any value above normal (according to the local laboratory)
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:10099.999.9
Pulse deficit or claudication
Four limbs BP discrepancy
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.