The recent death of John Travolta’s sonJetthas highlighted an uncommon disease known as Kawasaki disease. According to the Arthritis FoundationKawasaki disease is one of over 100 types of arthritis.
What is Kawasaki disease?
Kawasaki disease is a form of vasculitisor inflammation of the blood vessels. It typically develops in young children with over 80% of patients under the age of 5and 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 syndromeKawasaki’s diseaseKawasaki’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 infectionbut this has not yet been proven. There are also those that believe it is an autoimmune condition. In additionthere 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 clearsthere can still be cardiac complications that appear years later.
The symptoms include:
High-grade fever102 °F to 104 °Fthat lasts for more than 5 days if left untreated
Conjunctivitisalso know as “Pink Eye”
Chappedbright 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 nodesusually in the neck area
Other symptoms can include abdominal paindiarrheajaundice 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 additionKawasaki disease is one of the few cases where aspirin is used to treat children. Aspirin is given in high doses until the fever subsidesand 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 recoveralthough in a longer time frame than if treated. Howeverif untreated the risk of coronary artery disease is much higher.
Overallabout 2% of Kawasaki disease patients die from complications of coronary inflammation. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeksand 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. Alsotreatment with aspirin can increase the risk of bleeding and has been associated with the risk of Reye’s syndrome in children.