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Objectives: There are few data on clinical profiles of ANCA-associated vasculitis (AAV) in different ethnic populations. The aim of this study was to examine the differences in the ANCA type and clinical features of AAV between populations using the Diagnostic and Classification Criteria in Vasculitis Study (DCVAS) dataset. Methods: The DCVAS is an international, multicentre, observational study recruiting in 133 sites. Eight ethnic categories were analysed: Northern European, Caucasian American, Southern European, Middle Eastern/Turkish, Chinese, Japanese, Indian subcontinent and other. ANCA type was categorized as myeloperoxidase (MPO), PR3 and ANCA negative. Organ system involvement was recorded using a standard dataset. Differences were analysed by chi-squared tests using a Bonferroni correction and logistic regression (adjusting for age and sex). Northern European was the reference population. Results: Data from 1217 patients with AAV were available and the 967 (79.5%) patients recruited by rheumatology departments were analysed to reduce confounding by recruitment specialty. There were differences in ANCA type between ethnic categories (P < 0.001): MPO-ANCA was more common than PR3-ANCA in Japanese, Chinese and Southern Europeans; PR3-ANCA was more common in the other groups. Compared with Northern Europeans, Japanese had a nearly 60-fold increased chance of having MPO-ANCA (vs PR3-ANCA) [odds ratio (OR) 59.2 (95% CI 8.0, 440.7), P < 0.001] and Chinese had a nearly 7-times increased chance [OR 6.8 (95% CI 2.6, 17.8), P < 0.001]. Ophthalmologic and otorhinolaryngologic involvement were less common in Japanese and Chinese populations than Northern Europeans; otherwise, there were few differences in organ involvement between ethnic groups. Conclusion: This study confirms the previously observed differential occurrence of MPO-AAV and PR3-AAV between different ethnic groups.

Original publication




Journal article


Rheumatology (oxford)

Publication Date





1962 - 1969


ANCA-vasculitis, MPO-ANCA, PR3-ANCA, epidemiology, Aged, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antibodies, Antineutrophil Cytoplasmic, Arabs, Asian Continental Ancestry Group, China, Europe, European Continental Ancestry Group, Eye Diseases, Female, Female Urogenital Diseases, Heart Diseases, Humans, India, Japan, Kidney Diseases, Male, Male Urogenital Diseases, Middle Aged, Middle East, Myeloblastin, Nervous System Diseases, Odds Ratio, Otorhinolaryngologic Diseases, Peroxidase, Respiratory Tract Diseases, Skin Diseases, Turkey, United States