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Modification of 2022 ACR/EULAR ANCA-associated vasculitis classification criteria in a Japanese cohort (J-CANVAS)

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34

 

  1. Department of Rheumatology, University of Yamanashi Hospital, Yamanashi, Japan.
  2. Inflammation and Immunology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
  3. Department of Rheumatology, University of Yamanashi Hospital, Yamanashi, Japan.
  4. Department of Rheumatology, University of Yamanashi Hospital, Yamanashi, Japan.
  5. Inflammation and Immunology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
  6. Department of Internal Medicine and Rheumatology, Juntendo University, Tokyo, Japan.
  7. Center for Rheumatic Disease, Japanese Red Cross Society Kyoto Daiichi Hospital, Kyoto, Japan.
  8. Department of Rheumatology, Chubu Rosai Hospital, Nagoya, Japan.
  9. Immuno-Rheumatology Center, St. Luke's International Hospital, Tokyo, Japan.
  10. Department of Rheumatology, Japanese Red Cross Society Kyoto Daini Hospital, Kyoto, Japan.
  11. Department of Nephrology, Kyoto Katsura Hospital, Japan.
  12. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, Japan.
  13. Inflammation and Immunology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto; and Department of Hematology and Rheumatology, Kagoshima University Hospital, Japan.
  14. Department of General Internal Medicine, Tottori Prefectural Central Hospital, Japan.
  15. Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Japan.
  16. Department of Diabetes, Endocrinology and Clinical Immunology, Hyogo Medical University School of Medicine, Japan.
  17. Department of Internal Medicine (IV), Osaka Medical and Pharmaceutical University, Japan.
  18. Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences; and Department of Rheumatology, Shimane University Faculty of Medicine, Japan.
  19. Department of Rheumatology, Endocrinology and Nephrology, Graduate School of Medicine, Hokkaido University; and The First Department of Internal Medicine, University of Toyama Faculty of Medicine, Toyama, Japan.
  20. Division of Rheumatology, Department of Medicine, Showa University School of Medicine, Japan.
  21. Department of Rheumatology, Tokyo Kyosai Hospital; and Department of Rheumatology, Graduate School of Medical and Dental Sciences, Institute of Science Tokyo (formerly Tokyo Medical and Dental University), Japan.
  22. Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan.
  23. Department of Rheumatology, Graduate School of Medical Science, Kanazawa University, Japan.
  24. Division of Respiratory Medicine and Rheumatology, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori University, Japan.
  25. Division of Gastroenterology and Nephrology, Tottori University, Japan.
  26. Division of Nephrology, Department of Internal Medicine, Teikyo University Chiba Medical Center, Japan.
  27. Department of Rheumatology, Shimane University Faculty of Medicine, Japan.
  28. Rheumatic Disease Center, Sasebo Chuo Hospital; and Department of Public Health, Nagasaki University Graduate School of Biomedical Science, Japan.
  29. Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan.
  30. Division of Rheumatology, Department of Medicine, Showa University School of Medicine, Japan.
  31. The Clinical and Translational Research Center, University Hospital, Kyoto Prefectural University of Medicine, Japan.
  32. Department of Clinical Assessment, Tokyo University of Pharmacy and Life Sciences, Japan.
  33. Inflammation and Immunology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
  34. Department of Rheumatology, University of Yamanashi Hospital, Yamanashi, Japan. dnakagomi@hotmail.co.jp

CER19080
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PMID: 41231443 [PubMed]

Received: 07/07/2025
Accepted : 13/10/2025
In Press: 07/11/2025

Abstract

OBJECTIVES:
The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria (the 2022 ACR/EULAR criteria) for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were recently published. This study aimed to evaluate the accuracy of these criteria in Japanese patients with AAV using data from the Japan Collaborative Registry of ANCA-associated Vasculitis (J-CANVAS).
METHODS:
This multicentre retrospective cohort study analysed patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The 2022 ACR/EULAR criteria were applied to J-CANVAS participants to determine diagnostic performance, including sensitivity and specificity. A modified version of the 2022 ACR/EULAR criteria (the modified criteria) was subsequently developed, and its performance was compared with that of the 2022 ACR/EULAR criteria.
RESULTS:
In the J-CANVAS cohort, sensitivity values for MPA, GPA, and EGPA using the 2022 ACR/EULAR criteria were 96.1%, 48.5%, and 84.6%, respectively, while specificity values were 70.0%, 95.9%, and 98.0%, respectively. The modified criteria improved sensitivity to 90.7%, 83.8%, and 96.2% for MPA, GPA, and EGPA, respectively, and specificity to 86.7%, 99.0%, and 95.8%, respectively, with consistently higher AUC values.
CONCLUSIONS:
The modified criteria improved the accuracy of AAV classification in Japanese patients.

DOI: https://doi.org/10.55563/clinexprheumatol/5naes7

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