Kawasaki disease is a rare but serious illness that causes heart problems in children. It is a form of a condition called vasculitis (inflammation of the blood vessels). The blood vessels include arteries, veins, and capillaries.
Sometimes, the disease affects the coronary arteries that supply oxygenated blood to the heart.
If the disease affects the coronary arteries, it can lead to severe heart problems including myocarditis (inflammation of the heart muscle), dysrhythmia (abnormal heart rhythm), and aneurysm (weakening and bulging of the artery wall).
The disease can also affect the lymph nodes, the skin, and the mucous membranes found inside the nose, throat, and mouth.
Kawasaki disease was described by and named after Japanese pediatrician Tomisaku Kawasaki in 1967. It is also called mucocutaneous lymph node syndrome or Kawasaki syndrome.
Although Kawasaki disease was first described in Japan, the disease is found all over the world. It is more common in boys than in girls.
The Kawasaki Disease Foundation (KDF) estimates that Kawasaki disease affects more than 4,200 children in the United States each year (KDF).
According to a 1999 review published in American Family Physician, it is one of the primary causes of acquired heart disease in children in the United States and other developed countries (Taubert & Shulman, 1999).
Kawasaki disease can be alarming, but children will usually recover in a few weeks without developing serious heart problems if they are treated early. Relapses are uncommon. However, if the disease is not treated, it may lead to problems with the arteries of the heart and death due to a heart attack.
What Causes Kawasaki Disease?
The exact cause of Kawasaki disease is still unknown. Some studies suggest that it may be caused by the immune system’s reaction to an infectious agent, such as a virus. The condition itself is not contagious.
Who Is at Risk for Kawasaki Disease?
Kawasaki disease affects all ethnicities but is most common in children of Asian descent. Most patients are children under the age of 5.
What Are the Symptoms of Kawasaki Disease?
Kawasaki disease occurs in stages.
Early symptoms may include:
- high fever (often greater than 102 degrees and even as high as 104 degrees Fahrenheit) that persists for five or
- more days
- bloodshot eyes (also known as “conjunctivitis without discharge”)
- swollen, bright red tongue (referred to as a strawberry tongue)
- swollen lymph nodes
- swollen hands and feet
- red palms and soles of the feet
Later symptoms may include:
- peeling skin on the hands and feet (the skin may come off in sheets)
- pain in the joints
What are the less common findings?
Most of the common symptoms described above will resolve without complications, even if untreated.
Less common findings include inflammation of the lining of the heart (pericarditis), joints (arthritis), or covering of the brain and spinal cord (meningitis), and the other heart complications listed below in the section on serious complications.
The outlook (prognosis) for an individual affected is primarily determined by the seriousness of any heart complications.
What is Kawasaki’s disease, and how is it diagnosed?
Kawasaki’s disease is an uncommon illness in children that is characterized by high fever of at least five days’ duration together with at least four of the following five findings:
- Inflammation with reddening of the whites of the eyes (conjunctivitis) without pus
- Redness or swelling of the hands or feet, or generalized skin peeling
- Lymph node swelling in the neck
- Cracking, inflamed lips or throat, or red “strawberry” tongue
The above criteria are used to make a diagnosis of Kawasaki’s disease. The terminology “incomplete Kawasaki’s disease” is sometimes used for patients with only some features of classical Kawasaki’s disease.
Most patients are under 5 years of age.
What is the treatment for Kawasaki’s disease?
Children affected by Kawasaki’s disease are hospitalized. Kawasaki’s disease is treated with high doses of aspirin (salicylic acid) to reduce inflammation and to mildly thin the blood to prevent blood clot formation.
Also used in treatment is gammaglobulin administered through the vein (intravenous immunoglobulin or IVIG), together with fluids.
This treatment has been shown to decrease the chance of developing aneurysms in the coronary arteries. Sometimes cortisone medications are given.
Persisting joint pains are treated with anti-inflammatory drugs, such as ibuprofen (Advil) or naproxen (Aleve).
Plasma exchange (plasmapheresis) has been reported as effective in patients who were not responding to aspirin and gammaglobulin.
Plasmapheresis is a procedure whereby the patient’s plasma is removed from the blood and replaced with protein-containing fluids.
By taking out portions of the patient’s plasma, the procedure also removes antibodies and proteins that are felt to be part of the immune reaction that is causing the inflammation of the disease.
Kawasaki’s disease that is not responding to the traditional aspirin and gammaglobulin treatments can be deadly.
Medications that block the effects of TNF (tumor necrosis factor), one of the messenger molecules in the inflammatory response, are being studied for use in these situations.
Examples of TNF-blocking drugs are infliximab (Remicade) and etanercept (Enbrel). Further research is needed to design treatment programs for those who are failing conventional treatments. Pentoxifylline (Trental) is also being studied as a possible treatment for Kawasaki’s disease.