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

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).

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.

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.

The modified criteria improved the accuracy of AAV classification in Japanese patients.