Stages of Kawasaki Disease

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What is Kawasaki Disease?

Kawasaki disease (KD) was first reported in Japan in the 1960’s, and it is a disorder that affects mostly male Japanese children who are less than 5 years old. However, worldwide distribution has been reported, and it is known to affect many Koreans, blacks, Polynesians, Filipinos, Chinese, Australians and Americans.

Although the exact cause of the disease is unknown, it follows the pattern of an infectious disease and has been observed to occur as epidemics. It is also similar to an autoimmune disease since the disorder affects many organ systems including the skin, the muscles and joints, lungs, kidneys, the arteries and the heart, gastrointestinal and nervous systems.

What Are the Stages of Kawasaki Disease?

KD has three distinct stages:

The acute stage or stage I starts one to two weeks after the onset of the disease. It is characterized by high fever (temperature > 104° F) and irritability of the child. Most children have conjunctivitis (pink eyes) and reddening of the lips, tongue, hands and feet and the area around the anus. Skin eruptions like rashes and blisters are common. The lymph nodes in the neck are swollen. Other organs like the heart, the liver, the kidneys and the gastrointestinal system may also be involved. There are inflammatory changes in the blood vessels. This stage lasts for 1-11 days. Death may occur as a result of myocarditis or inflammation of the heart muscles.

The subacute stage or stage II starts two to four weeks after onset. Fever subsides but irritability persists. Children may experience lack of appetite, abdominal pain, vomiting and diarrhea. Inflammatory changes decrease but aneurysms may form in the blood vessels. Different cells in the blood proliferate. Peeling of the skin of the hands and feet are prominent. This stage lasts for about 11-30 days. Death may occur as a result of ischemic heart disease, rupture of an aneurysm or myocarditis.

The convalescent stage is the third stage. It starts four to seven weeks from onset and may last for more than a month. Inflammatory changes subside but thickening of the walls of blood vessels occur. Aneurysms may enlarge and thicken but some decrease in size. Death can occur as a result of ischemic heart disease, myocardial infarction (heart attack), rupture of an aneurysm or myocarditis.

Some authors include a fourth stage or chronic stage which occurs seven weeks after onset. It is the period of scar formation, thickening of the walls of the arteries and decrease in their diameters. Formation of scar tissues may also occur in the heart muscles. These changes are seen until the children develop into adults and are often found in autopsies after death usually resulting from a heart attack.

In general death from KD is uncommon (about 0.1-2%). Sudden death can occur because of complications involving the heart and aneurysms in the arteries. However, most of the inflammatory changes subside with adequate treatment and recurrence is low.


eMedicine, “Kawasaki Disease” accessed 2/11/11

Kawasaki, Tomisaku. Kawasaki Disease. Blackwell Publishing, accessed 2/11/11.