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Global ethnic and geographic differences in the clinical presentations of anti-neutrophil cytoplasm antibody-associated vasculitis

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Pearce FA1,2, Craven A3, Merkel PA4, Luqmani RA3, Watts RA5,6. Rheumatology (Oxford). 2017 Nov 1;56(11):1962-1969. doi: 10.1093/rheumatology/kex293.


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1 Division of Epidemiology and Public Health, University of Nottingham.

2 Department of Rheumatology, Nottingham University Hospital NHS Trust, Nottingham.

3 Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.

4 Division of Rheumatology and Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PA, USA.

5 Norwich Medical School, University of East Anglia, Norwich.

6 Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK.


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.