the contribution of genetic variation and infection to the pathogenesis of anca-associated systemic vasculitis遗传变异的贡献和感染anca-associated系统性血管炎的发病机制.pdf

Willcocks et al. Arthritis Research Therapy 2010, 12:202 /content/12/1/202 R E V I E W The contribution of genetic variation and infection to the pathogenesis of ANCA-associated systemic vasculitis 1 1 2,3 1 Lisa C Willcocks , Paul A Lyons , Andrew J Rees and Kenneth GC Smith* WG characteristically has ear, nose and throat and/or Abstract respiratory involvement. Necrotising granulomas may The aetiology of anti-neutrophil cytoplasmic antibody cause sinusitis, nasal discharge, damage to the nasal (ANCA)-associated systemic vasculitis has not been septum, hearing loss and/or haemoptysis. CSS is asso- well dei ned. Here we review two factors which may ciated with asthma and eosinophilia. MPA commonly play a role in the pathogenesis of the disease: genetics affects the kidney without evidence of granulo mas, upper and infection. In particular, we discuss the role of respiratory tract involvement or asthma. autoantibodies to LAMP-2, which may arise following The association with ANCA also varies: 80 to 90% of infection with Gram-negative bacteria, and may individuals with WG and MPA are ANCA-positive, contribute to the development of ANCA-associated compared with 40% of patients with CSS. The sensitivity systemic vasculitis in genetically susceptible individuals. for ANCA in the diagnosis of WG and MPA is 81 to 85%,