Kawasaki Disease
Also known as: mucocutaneous lymph node syndrome
What is it?
Kawasaki disease (also called mucocutaneous lymph node
syndrome) is a potentially fatal inflammatory disease
affecting several organ systems in the body, including the
heart, circulatory system, mucous membranes, skin, and
immune system.
Who gets it?
Kawasaki disease occurs primarily in infants and
children between the ages of 18 months and two years, but
is also diagnosed in adults as old as 34 years. Children
of Japanese and Korean descent have a higher incidence of
Kawasaki disease, but it can affect all racial and ethnic
groups. Approximately 2,000 cases occur each year in the
United States, with twice as many boys affected as girls.
What causes it?
Doctors haven’t been able to identify the cause or
causes of Kawasaki disease. It may be an allergic reaction
or other unusual response to certain types of infections.
Some researchers think the disease may be caused by the
interaction of an immune cell, called the T cell, with
certain poisons (toxins) secreted by bacteria. Recent
studies suggest the disease may be caused by an inhaled
pathogen that produces the same kind of targeted
antigen-antibody reactions that characterize other
infectious illnesses. Because it's rare for more than one
child in the same family to develop Kawasaki disease, it
does not appear to be hereditary.
What are the symptoms?
The first symptom of Kawasaki disease is a fever that's
often higher than 104 degrees Fahrenheit (39 degrees
Celsius), which is present for at least 5 days. Within a
day or 2, conjunctivitis appears in both eyes, causing
redness without the discharge usually associated with it.
After a few more days, a rash often appears in the trunk
region and the genital area. Lips become cracked, red, and
dry, and the tongue gets red and swollen like a
strawberry, with a white coating and prominent red bumps.
The throat may be sore and irritated as well. The palms of
the hands and soles of the feet start to look swollen,
purple-red, and irritated. About half of infected children
will also have at least one swollen lymph node in the
neck. During the second phase of the illness, the skin on
the hands and feet begins to peel off, often in large
sheets, revealing new skin underneath. Some children may
also experience joint pain, diarrhea, vomiting, or
abdominal pain. During the third phase, the symptoms
gradually subside unless complications develop. A few
patients develop symptoms of inflammation in the liver
(hepatitis), gallbladder, lungs, or tonsils, and some
develop an inflammation of the membranes covering the
brain and spinal cord (meningitis). A small percentage of
patients with Kawasaki disease develop complications of
the cardiovascular system. These complications include
inflammation of the heart tissue (myocarditis),
disturbances in heartbeat rhythm (arrhythmias), and areas
of blood vessel dilation (aneurysms) in the coronary
arteries. Other patients may develop inflammation of an
artery (arteritis) in their arms or legs.
How is it diagnosed?
There is no specific test available to diagnose
Kawasaki disease conclusively. Therefore, a doctor may
observe a child's symptoms and rule out other diseases
that could be causing them. Once other diseases have been
ruled out, the patient's symptoms will be compared with a
set of diagnostic criteria. The patient must have a fever
lasting five days or longer that does not respond to
antibiotics, together with four of the following five
symptoms:
- conjunctivitis in both eyes without thick discharge
- changes around the lips, tongue, or mouth
- changes in the fingers and toes, such as swelling,
discoloration, or peeling
- a rash in the trunk or genital area
- a large swollen lymph node (or nodes) in the neck
As soon as doctors suspect Kawasaki disease - sometimes
even before all the symptoms are present - they typically
order an evaluation of the heart, including an
echocardiogram. If a child has just two or three of the
above symptoms but this test reveals coronary artery
abnormalities, then Kawasaki disease is usually diagnosed.
How is it treated?
Treatment for Kawasaki disease should be started as
soon as possible, ideally within 10 days of the onset of
illness. Usually, treatment consists of the patient
receiving purified antibodies given intravenously over 12
hours, or smaller doses given over 4 days. A doctor may
prescribe a high-dose aspirin to reduce the risk of
coronary artery aneurysms. If a coronary aneurysm is
present, high-dose aspirin may be continued for up to 8
weeks or until an echocardiogram shows improvement. A
small number of children may also need other forms of
blood thinners to keep clots from forming.
Self-care tips
For patients being treated for Kawasaki disease,
follow-up care may include two to three months of
monitoring with chest x rays, electrocardiography, and
echocardiography. Treatment with aspirin is often
continued for several months.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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