The recent death of John Travolta’s son, Jett, has highlighted an uncommon disease known as Kawasaki disease. According to the Arthritis Foundation, Kawasaki disease is one of over 100 types of arthritis.
What is Kawasaki disease?
Kawasaki disease is a form of vasculitis, or inflammation of the blood vessels. It typically develops in young children with over 80% of patients under the age of 5, and affects boys more than girls.
Kawasaki disease was first identified by Tomasaku Kawasaki in Japan in 1967. It was also known as mucocutaneous lymph node syndrome, Kawasaki’s disease, Kawasaki’s syndrome and infantile polyarteritis.
Kawasaki disease is considered in the U.S. to be the leading cause of acquired heart disease in children.
What is the cause of Kawasaki disease?
The cause of Kawasaki disease has not been discovered. There are theories that it is the result of an infection, but this has not yet been proven. There are also those that believe it is an autoimmune condition. In addition, there appears to be a genetic link to development of the disease.
What are the symptoms of Kawasaki disease?
Kawasaki disease develops quickly and is very aggressive for several weeks. After the disease clears, there can still be cardiac complications that appear years later.
The symptoms include:
High-grade fever, 102 °F to 104 °F, that lasts for more than 5 days if left untreated
Conjunctivitis, also know as “Pink Eye”
Chapped, bright red lips
Red mucous membranes in the mouth
Strawberry looking tongue
Joint pain and swelling on both sides of the body
Rapid heart beat
Peeling skin on the palms and soles of the feet
Palms of the hands and soles of the feet are red
Swollen lymph nodes, usually in the neck area
Other symptoms can include abdominal pain, diarrhea, jaundice and meningitis.
What is the treatment for Kawasaki disease?
Children should be hospitalized and treated by a physician experienced in treating Kawasaki disease. The usual course of treatment includes intravenous immunoglobulin (IVIG) given in high doses. In addition, Kawasaki disease is one of the few cases where aspirin is used to treat children. Aspirin is given in high doses until the fever subsides, and then in low doses for two to three months to prevent blood clotting.
What is the prognosis?
If the disease is promptly diagnosed and treatment started quickly the recovery is generally rapid. If left untreated the patient will usually recover, although in a longer time frame than if treated. However, if untreated the risk of coronary artery disease is much higher.
Overall, about 2% of Kawasaki disease patients die from complications of coronary inflammation. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every 1–2 years to screen for progression of cardiac involvement.
It is also possible that the patient may experience a relapse of the symptoms shortly after treatment with IVIG which would require hospital treatment again. Also, treatment with aspirin can increase the risk of bleeding and has been associated with the risk of Reye’s syndrome in children.