With treatment, the risk of death is reduced to 0.17%. People who have had coronary artery aneurysms after Kawasaki disease require lifelong cardiological monitoring by specialized teams.Kawasaki disease is rare. It affects between 8 and 67 per 100,000 people under the age of five except in Japan, where it affects 124 per 100,000. Boys are more commonly affected than girls. The disorder is named after Japanese pediatrician Tomisaku Kawasaki, who first described it in 1967. Signs and symptoms
Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol (acetaminophen) or ibuprofen. This is the most prominent symptom of Kawasaki disease, and is a characteristic sign that the disease is in its acute phase; the fever normally presents as a high (above 39–40 °C) and remittent, and is followed by extreme irritability. Recently, it is reported to be present in patients with atypical or incomplete Kawasaki disease; nevertheless, it is not present in 100% of cases.The first day of fever is considered the first day of the illness, and its duration is typically one to two weeks; in the absence of treatment, it may extend for three to four weeks. Prolonged fever is associated with a higher incidence of cardiac involvement. It responds partially to antipyretic drugs and does not cease with the introduction of antibiotics. However, when appropriate therapy is started – intravenous immunoglobulin and aspirin – the fever subsides after two days.Bilateral conjunctival inflammation has been reported to be the most common symptom after fever.
Infectious conditions that can mimic Kawasaki disease include periorbital cellulitis, peritonsillar abscess, retropharyngeal abscess, cervical lymphadenitis, parvovirus B19, mononucleosis, rheumatic fever, meningitis, staphylococcal scalded skin syndrome, toxic epidermal necrolysis, and Lyme disease. Kawasaki-like disease temporally associated with COVID-19
In 2020, reports of a Kawasaki-like disease following exposure to SARS-CoV-2, the virus responsible for COVID-19, emerged in the US and Europe. The World Health Organization is examining possible links with COVID-19. This emerging condition was named paediatric multisystem inflammatory syndrome by the Royal College of Paediatrics and Child Health, and multisystem inflammatory syndrome in children by the Centers for Disease Control and Prevention. Guidance for diagnosis and reporting of cases has been issued by these organizations. Classification
Debate has occurred about whether Kawasaki disease should be viewed as a characteristic immune response to some infectious  pathogen, as an autoimmune process, or as an autoinflammatory disease (i.e. involving innate rather than adaptive immune pathways). Overall, immunological research suggests that Kawasaki disease is associated with a response to a conventional antigen (rather than a superantigen) that involves both activation of the innate immune system and also features of an adaptive immune response. Identification of the exact nature of the immune process involved in Kawasaki disease could help guide research aimed at improving clinical management.Inflammation, or vasculitis, of the arteries and veins occurs throughout the body, usually caused by increased production of the cells of the immune system to a pathogen, or autoimmunity.