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Kawasaki disease is a type of vasculitis, which means inflammation of the blood vessels. This month’s JAMA Pediatrics includes a review article summarizing what we have learned about Kawasaki disease over the past 50 years since it was first identified.
Kawasaki disease most commonly affects children between the ages of 6 months and 5 years; boys are 50% more likely than girls to get this illness. Although the cause of Kawasaki disease remains unknown, it is likely that this disease is a final common pathway of many infectious or environmental factors that trigger inflammation of the blood vessels in people who have a genetic predisposition to get this disease.
Kawasaki disease has 6 diagnostic criteria, which include fever persisting for 5 or more days, in addition to 4 of 5 signs of inflammation in specific areas that are often called “mucocutaneous areas” because they involve the skin or mucous membranes, such as the eyes or mouth:
Inflammation of the blood vessels in the eye, also called conjunctival injection, conjunctivitis, or “pink eye.”
Inflammation of the blood vessels in the mouth and throat, which can be seen as cracked lips, a red tongue (often called a “strawberry tongue”), or a red throat.
Inflammation of the blood vessels in the hands or feet, often seen as redness on the palms or soles of the feet, swelling of the hands or feet, or shedding of skin on the hands or feet.
Rash anywhere on the body.
Swollen lymph nodes in the neck, with at least 1 lymph node more than 1.5 cm in diameter.
In some cases, an echocardiographic examination of the arteries around the heart (coronary arteries) is helpful to both diagnose Kawasaki disease and determine the type of treatment needed. The most serious concerns in Kawasaki disease are the inflammation of the coronary arteries and its potential effects on the heart. Other consequences can include inflammation of vessels in the gastrointestinal system leading to problems in the liver or gallbladder, inflammation of vessels in the brain leading to meningitis, or inflammation of vessels in the eye or ear leading to sensory problems.
Treatment includes intravenous gamma globulin, which helps reduce inflammation in the vessels and usually results in rapid improvement in symptoms. If vessels in the heart are affected, additional treatments such as anticoagulation medications and steroids are sometimes used. Fortunately, most children with Kawasaki disease recover completely. Five percent of children who had coronary artery involvement with Kawasaki disease also had persistent abnormalities, although these abnormalities affected only 25% of these children. If you suspect your child may have Kawasaki disease, see your pediatrician.
American Academy of Pediatrics
Correction: This article was corrected on February 27, 2017, to fix an error in the figure.
Moreno MA. Summarizing What Has Been Learned About Kawasaki Disease. JAMA Pediatr. 2016;170(11):1124. doi:10.1001/jamapediatrics.2015.2568
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