Epidemiology of systemic vasculitis.

Purpose of review: Ongoing research contributes to our understanding of the epidemiology of vasculitis and its outcomes across the globe. This review aims to summarize important research studies published on this topic in the last 18 months.

Recent findings: The implementation of rapid referral systems and use of large vessel imaging have improved the diagnosis of giant cell arteritis. A population-based study in immunoglobulin G4-related disease provides incidence and prevalence estimates for the United States for the first time. Recently published data supported viral infectious triggers for Kawasaki disease and immunoglobulin A vasculitis. Population studies in antineutrophil cytoplasmic antibody associated vasculitis report an increase in the incidence of eosinophilic granulomatosis with polyangiitis and have provided further insights into the burden of cardiovascular disease in these patients. Data on Behçet's disease continues to show increased all-cause mortality and need for better treatment strategies.

Summary: Recent literature highlights the heterogeneity of the epidemiology of vasculitis in different parts of the world as well as associated outcomes, comorbidities, and potential triggers. Thought new classification criteria are being employed in some forms of vasculitis, standardization of case identification remains an unmet need in multiple other forms of vasculitis.