Current Concept and Epidemiology of Systemic Vasculitides

Although a new classification algorithm for systemic vasculitides was proposed by Watts et al. and the Chapel Hill Consensus Conference (CHCC) was updated in 2012, there are currently no validated diagnostic criteria for systemic vasculitides. The Diagnostic and Classification Criteria for Vasculitis study (DCVAS) is a global study to develop and improve the diagnostic criteria for systemic vasculitides. The epidemiology of systemic vasculitides differs widely among countries. For example, in the case of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, patients with microscopic polyangiitis (MPA) and with positivity for MPO-ANCA are predominant in Asian countries, whereas patients with granulomatosis with polyangiitis (GPA) and with positivity for PR3-ANCA are predominant in northern Europe and the United States. Interstitial lung disease (ILD) occurs more frequently in Asian patients compared with patients in Europe. The incidence and the prevalence of large-vessel vasculitis also differ significantly. Giant cell arteritis (GCA) occurs frequently in northern Europe, unlike Takayasu arteritis (TAK). The ethnic and regional differences in the incidence, prevalence and clinical characteristics of patients with vasculitis should be recognized when we diagnose and treat patients with vasculitis using criteria, and should also be considered when interpreting the results from clinical studies.