Abstract

ObjectiveTo investigate the association of farm exposure and the development of ANCA-associated vasculitis (AAV).MethodsOne hundred eighty-nine well defined patients with AAV (n = 119 with granulomatosis with polyangiitis [GPA], n = 48 with microscopic polyangiitis [MPA], n = 22 patients with eosinophilic granulomatosis with polyangiitis [EGPA]) and 190 controls (n = 119 patients with rheumatoid arthritis, n = 71 with large vessel vasculitis) were interrogated using a structured questionnaire. Factors investigated were occupation, farm exposure, contact to different livestock, participation in harvesting, residence next to a farm, MRSA status, and contact to domestic pets at disease onset or ever before. The odds ratio (OR) and 95% confidence interval [95%CI] were calculated for each item.ResultsUnivariate analysis revealed a strong association of AAV with regular farm exposure; OR 3.44 [95%CI 1.43–8.27]. AAV was also associated with regular contact to cattle 4.30 (1.43–8.27), pigs 2.75 (1.12–6.75) and MRSA carriage 3.38 (1.11–10.3). This association was stronger in the subgroup of GPA patients. OR in this group for farm exposure was 4.97; [2.02–12.2], for cattle 6.71 [95% CI 2.19–20.7], for pigs 4.34 [1.75–10.9], and MRSA carriage 5.06 [1.62–15.8]). There was no significant association of MPA or EGPA with these parameters.ConclusionA significant association between farm exposure or farm animal exposure and AAV especially in the subgroup of patients with GPA has been identified. This suggests that these entities are distinct and have different triggers for the immune process.

Highlights

  • Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotising vasculitis predominantly affecting small vessels

  • Univariate analysis revealed a strong association of AAV with regular farm exposure; odds ratio (OR) 3.44 [95% confidence intervals (95%CI) 1.43–8.27]

  • GPA was diagnosed according to the American College of Rheumatology (ACR) criteria and the criteria adapted from the 2012 revised Chapel Hill Consensus Conference (CHCC) [1, 15]

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Summary

Introduction

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotising vasculitis predominantly affecting small vessels. AAV comprises granulomatosis with polyangiits (GPA, Wegener’s), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) [1]. The annual incidence of AAV as a group is estimated at about 10–20 patients per million [2]. The causes of AAV are poorly understood and their pathophysiological mechanisms remain uncertain [3]. In a recent genome-wide association study of patients with AAV a genetic contribution to the pathogenesis has been confirmed [4]. Beside the genetic susceptibility microbial pathogens and environmental factors have been implicated in the pathogenesis of AAV [5]

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