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.

Abstract

Vasculitis, inflammation of the blood vessels, takes many forms and can affect every organ system, potentially causing kidney failure, vision loss and death. This Review describes current knowledge on the global incidence and prevalence patterns for various types of vasculitis.

Key points

• Vasculitis is a group of generally uncommon diseases of generally unknown aetiology.

• Vasculitis mainly occurs at the ends of the age spectrum, in young children and older adults.

• Kawasaki disease is most common in children aged 5 years or less and occurs most commonly in Japan and in children of Southeast Asian ancestry.

• Giant cell arteritis is the most common form of vasculitis in elderly individuals, particularly in those of Northern European ancestry.

• The three types of antineutrophil cytoplasmic antibody-associated vasculitis are rare; of the three types, granulomatosis with polyangiitis is the most prevalent.

• Behçet syndrome has the highest prevalence along the ancient silk road, which stretches from the Mediterranean through the Middle East to East Asia.