Global epidemiology of vasculitis

Abstract

The many forms of vasculitis are characterized by inflammation of blood vessels, leading to potentially long-term sequelae including vision loss, aneurysm formation and kidney failure. Accurate estimation of the incidence and prevalence has been hampered by the absence of reliable diagnostic criteria and the rarity of these conditions; however, much progress has been made over the past two decades, although data are still lacking from many parts of the world including the Indian subcontinent, China, Africa and South America. Giant cell arteritis occurs in those aged 50 years and over and seems to mainly affect persons of northern European ancestry, whereas Takayasu arteritis occurs mainly in those aged under 40 years. By contrast, Kawasaki disease mainly occurs in children aged under 5 years and is most common in children of Asian ancestry, and IgA vasculitis occurs in children and adolescents. Although much less common than giant cell arteritis, the different forms of antineutrophil cytoplasmic antibody-associated vasculitis are being increasingly recognized in most populations and occur more frequently with increasing age. Behçet syndrome occurs most commonly along the ancient silk road between Europe and China. Much work needs to be done to better understand the influence of ethnicity, geographical location, environment and social factors on the development of vasculitis.

Fig. 1. Global incidence of giant cell arteritis.

a | Giant cell arteritis occurs most frequently in populations of Northern European ancestry. See Supplementary Table 1 for data used. Grey indicates no data available. b | Changing incidence of giant cell arteritis. Giant cell arteritis is gradually becoming less common, having peaked in incidence around 1990. Data from refs^,–.

Fig. 2. Global incidence of Kawasaki disease.

Kawasaki disease occurs most frequently in East Asia, especially Japan, South Korea and China, with a relatively equal distribution elsewhere. See Supplementary Table 2 for data used. Grey indicates no data available.

Fig. 3. Global incidence of ANCA-associated vasculitis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis subtypes show different patterns of occurrence across populations. For example, in Southern Europe and Japan, microscopic polyangiitis is more common than granulomatosis with polyangiitis, whereas as in most other populations, granulomatosis with polyangiitis is the more common form. Eosinophilic granulomatosis with polyangiitis is the least common of the three conditions. See Supplementary Table 3 for data used. Grey indicates no data available. a | The global incidence of granulomatosis with polyangiitis. b | The global incidence of microscopic polyangiitis. c | The global incidence of eosinophilic granulomatosis with polyangiitis.

Fig. 4. Global prevalence of Behçet syndrome.

Behçet syndrome occurs most commonly along the ancient silk road between the Mediterranean and China. See Supplementary Table 4 for data used. Grey indicates no data available.