Epidemiology of Kawasaki Disease in Europe

Aim of the review: To review major epidemiological aspects of Kawasaki disease (KD) in Europe, describing demographic characteristics, revising its incidence along with time trends and geographic variations, and describing migration studies to provide clues about its etiology.

Recent findings: The annual incidence of KD in Europe is about 10–15 per 100,000 children under 5 years old and seems to be relatively stable over time and space. Demographic characteristics are in line with those in other countries of the world, with a higher incidence in children from Asia and possibly North African origin. All studies performed across Europe found a coherent seasonal distribution of KD onset peaking from winter to early spring. This seasonal distribution was consistent over the years and suggests a climate-related environmental trigger. The occurrence of peaks during pandemics, microbiological findings and a possible link with southerly winds support the hypothesis of an airborne infectious agent. Neither other airborne agents such as pollutants or pollens nor urbanization and industrialization seem to have major effect on the etiology.

Conclusion: Discrepancies in KD incidence rates across studies were due more to methodological differences, variation in definitions and awareness of the disease than a real increase in incidence. Genetic predisposition is undeniable in KD, but environmental factors seem to play a pivotal role. Several lines of evidence support a non-exclusive airborne infectious agent with a protective immune response by the host as a key factor in inducing the inflammatory cascade responsible for symptoms and complications.