Abstract

The present study validates the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria for Takayasu's arteritis (TAK) compared with 1990 ACR TAK classification criteria. The fulfillment of 2022 ACR/EULAR and 1990 ACR TAK criteria from four referral centers was assessed for TAK compared with extracranial giant cell arteritis (EC-GCA) and other controls. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), likelihood ratio of a positive test (LR+) or negative test (LR-), and area under receiver operating characteristics curve (AUC) were calculated. Among 504 TAK (404 females) and 222 controls (151 females, 144 EC-GCA), the 2022 ACR/EULAR criteria had better sensitivity (95.83% vs 82.94%) and NPV but poorer specificity (63.51% vs 90.54%), PPV, LR+, LR-, and AUC at the pre-determined cut-offs than 1990 ACR criteria. The 2022 ACR/EULAR criteria had greater specificity (76.06% vs 57.62%) and AUC (0.845 vs 0.771) with similar sensitivity (93% vs 96.53%) in males than in females. The 2022 ACR/EULAR criteria performed similarly with only EC-GCA as controls (sensitivity 95.83%, specificity 60.42%, AUC 0.781). Sensitivity remained similar, whereas specificity was higher for 40-60 vs < 40 years. Cut-offs ≥6 (sensitivity 91.87%, specificity 82.88%) and ≥7 (sensitivity 86.71%, specificity 86.49%), or removing the point for female sex (sensitivity 92.64%, specificity 81.08%) greatly improved balance between sensitivity and specificity. The poor specificity of the 2022 ACR/EULAR TAK criteria in real-life settings was improved by increasing the cut-off to 6 or 7 or removing the point for female sex.

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