Validation and clinical application of 2022 ACR/EULAR criteria for Takayasu arteritis in a large Chinese cohort

Jinwei Gao; Shiping He; Yao Liu; Shuning Guo; Jing Wang; Chenglong Fang; Lingyu Liu; Yuan Li; Lili Pan; Xinwang Duan; Lijun Wu; Hongbin Li; Hongfeng Zhang; Xiaofeng Zeng; Jing Li; Xinping Tian

doi:10.1093/rheumatology/keaf372

Validation and clinical application of 2022 ACR/EULAR criteria for Takayasu arteritis in a large Chinese cohort

Rheumatology (Oxford). 2025 Jul 11:keaf372. doi: 10.1093/rheumatology/keaf372. Online ahead of print.

Authors

Jinwei Gao¹, Shiping He^{2

3}, Yao Liu⁴, Shuning Guo¹, Jing Wang^{5

6}, Chenglong Fang⁷, Lingyu Liu⁸, Yuan Li¹, Lili Pan⁹, Xinwang Duan¹⁰, Lijun Wu¹¹, Hongbin Li¹², Hongfeng Zhang¹³, Xiaofeng Zeng¹, Jing Li¹, Xinping Tian¹

Affiliations

¹ Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
² Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.
³ Department of Rheumatology and Immunology, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China.
⁴ Department of Rheumatology and Immunology, Loudi Central Hospital, Loudi, China.
⁵ Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
⁶ China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
⁷ Department of Rheumatology and Immunology, the Second Affiliated Hospital of Fujian Medical University, Quanzhou, China.
⁸ Department of Rheumatology and Immunology, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China.
⁹ Department of Rheumatology, Capital Medical University Affiliated Anzhen Hospital, Beijing, China.
¹⁰ Department of Rheumatology, the Second Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, China.
¹¹ Department of Rheumatology and Immunology, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjian, China.
¹² Department of Rheumatology, the Affiliated Hospital of Inner Mongolia Medical College, Hohhot, Inner Mongolia, 010050, China.
¹³ Department of Rheumatology, the First Hospital of Dalian Medical University, Dalian, Liaoning, China.

PMID: 40644612
DOI: 10.1093/rheumatology/keaf372

Abstract

Objective: To validate the performance of the 2022 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for Takayasu arteritis (TAK) in a China cohort and examine its performance in clinical practices.

Methods: 778 patients with TAK and 378 patients with non-TAK were included. The sensitivity, specificity, positive and negative predictive values, accuracy, and AUC of the 2022 ACR/EULAR and 1990 ACR criteria were assessed. Furthermore, the Kaplan-Meier curve was used to analyze the impact of high and low scores on prognosis according to the 2022 ACR/EULAR criteria for TAK.

Results: In this Chinese cohort, the 2022 ACR/EULAR criteria demonstrated superior performance with a sensitivity of 95.2% (95% CI: 93.4%-96.5%), specificity of 95.8% (93.1%-97.5%), and an AUC of 0.955 (0.940-0.970), compared with the 1990 ACR criteria, which had a sensitivity of 93.6% (91.6%-95.1%), specificity of 92.3% (89.0%-94.7%), and an AUC of 0.930 (0.911-0.948). Based on the 2022 ACR/EULAR criteria, false-negative cases had fewer vascular ischaemia symptoms and less affected arterial regions than true positives, while false positives mainly differed in angina and blood pressure. Survival analysis indicated that patients with scores ≥15 had significantly lower overall survival rates than those with scores <15, highlighting the need for increased clinical attention to these patients.

Conclusion: Compared with the 1990 ACR criteria, the 2022 ACR/EULAR criteria for TAK demonstrate better specificity and sensitivity in real-world clinical practice. The score derived from the 2022 classification criteria is related to the prognosis.

Keywords: Observational Studies; Rare diseases; Takayasu’s disease; Validation Study; Vasculitis.

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