A new study has compared the Ministry of Health, Labour, and Welfare (MHLW) diagnostic criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with the new American College Rheumatology (ACR)/ European Alliance of Associations for Rheumatology (EULAR) 2022 criteria.

The study, published in Modern Rheumatology, found the criteria generally in agreement and useful for categorizing patients into one of the 3 main AAV diseases.

“As a result of the diagnostic and classification criteria for vasculitis (DCVAS) study, the EULAR recently proposed new classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA),” the authors wrote. “In this study, we applied the MHLW criteria as well as the new ACR/EULAR criteria to patients with AAV in Japan who were enrolled in two previous prospective cohort studies, and investigated the reasons for the discordance between the two criteria.”

The research team analyzed data from 2 Japanese prospective cohort series comprising 22 university and referring hospitals and 53 institutions treating 156 patients with AAV. Medical history, eosinophilia, glomerulonephritis, ANCA phenotype, histology, and Birmingham Vasculitis Activity Score were collected.

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Patients were reclassified based on the NHLW and ACR/EULAR 2022 criteria for AAV, and the sensitivity and specificity of the NHLW criteria were calculated based on ACR/EULAR 2022 criteria as a reference.

The authors note that comparing the 2 methods was challenging because 1 set was developed for clinical research whereas the other was established to define patient eligibility for medical expense coverage.

However, the results showed that, according to the MHLW criteria, 71.3% of patients met at least 2 criteria, and only 2.1% were unclassifiable. Most patients could be classified into EGPA, GPA, or MPA, and the classification was relatively consistent with the ACR/EULAR 2022 criteria, particularly when the order of criteria application was considered.


Sada K, Nagasaka K, Kaname S, et al. Evaluation of Ministry of Health, Labour and Welfare diagnostic criteria for antineutrophil cytoplasmic antibody-associated vasculitis compared to ACR/EULAR 2022 classification criteria. Mod Rheumatol. Published online June 3, 2023. doi:10.1093/mr/road052