Prevalence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and spatial association with quarries in a French Northeast region.

Objective Silica is one of the strongest environmental substances linked with autoimmunity. The aim of this study was to assess the prevalence of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and renal limited vasculitis (RLV) in a French northeast region, and their geospatial association with quarries. Methods Potential ANCA-Associated Vasculitis (AAV) cases were identified using three sources: hospital records, immunology laboratories and the National Health Insurance System. Patients who resided in Alsace on January 1, 2016 and fulfilled ACR AAV criteria or Chapel Hill Consensus Conference 2012 definition were included. Incomplete case ascertainment was corrected using capture-recapture analysis. The spatial association between the number of cases and quarries in each administrative entity was assessed using geographical weighted regression (GWR). Results From 910 potential AAV cases, we identified 185 patients meeting inclusion criteria: 120 GPA, 35 MPA, 30 RLV. The number of cases missed by any source was 6.4 (95%CI 3.6-11.5). Accordingly, the 2016 estimated prevalence in Alsace was 65.5 cases per million inhabitants (95%CI 47.3-93.0) for GPA, 19.1 (95%CI 11.3-34.3) for MPA, and 16.8 (95%CI 8.7-35.2) for RLV. The risk of AAV was significantly increased in communes with quarries (OR: 2.51 [95%CI: 1.66-3.80]) and GWR revealed a significant spatial association between quarries and GPA cases (p = 0.039), and, regarding ANCA serotype, between quarries and both PR3-AAV (p = 0.04) and MPO-AAV (p = 0.03)." Conclusion In a region with a high density of quarries, the spatial association of AAV with quarries supports the role of silica as a specific environmental factor.