The epidemiology of systemic vasculitis is becoming increasingly well understood.
Giant cell arteritis is the commonest type of vasculitis with an incidence that is
highest in populations of Scandinavian descent, where the annual incidence reaches
15 to 35/100,000 aged > 50 years. Takayasu's arteritis has a relatively uniform global
incidence of one to two/million. The ANCA-associated vasculitides have an overall
incidence of 20/million with a peak age of onset at 65 to 74 years. Wegener's granulomatosis
appears to be more common in northern Europe compared with microscopic polyangiitis,
which seems to be more common in southern Europe. Henoch-Schönlein purpura is the
commonest form of childhood vasculitis in the West with an incidence of 20/100,000
aged < 17 years, but it is much rarer in adults (13/million). Kawasaki disease is
commonest in the childhood population of southeast Asia; in Japan the incidence is
500/million aged < 5 years, 50% of cases occur in those aged < 2 years. Behçet's disease
occurs along the Silk Road and in the Mediterranean littoral with a prevalence in
Turkey of 380/100,000. The various types of vasculitis have very different geographical
and ethnic distributions, which provide clues to the pathogenesis.
KEYWORDS
Vasculitis - epidemiology - Wegener's granulomatosis - giant cell arteritis - microscopic
polyangiitis
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Richard A WattsD.M. F.R.C.P.
Department of Rheumatology, Ipswich Hospital NHS Trust
Heath Road, Ipswich
IP4 5PD, UK
Email: Richard.watts@ipswichhospital.nhs.uk
