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AHA revises guidelines on Kawasaki disease
Oct. 25, 2004
The revised American Heart Association (AHA) guidelines on Kawasaki disease, published in the Oct. 26, 2004, issue of Circulation, provide recommendations to guide the clinician in deciding which children to treat for Kawasaki disease. This statement is also endorsed by the American Academy of Pediatrics and copublished in the upcoming Dec. 1, 2004, issue of Pediatrics. "In the past, physicians have relied on the classic criteria to decide which children should be treated for Kawasaki disease," lead author Jane W. Newburger, MD, MPH, from Harvard University and Children's Hospital in Boston, Massachusetts, says in a news release. "We now understand that some children with Kawasaki disease never fulfill these classic criteria, yet are at risk for developing coronary artery aneurysms."

Kawasaki disease is an acute inflammation of blood vessels and the leading cause of acquired heart disease in children in the U.S., affecting more than 4,000 children each year. More than 80% of children who are diagnosed as having Kawasaki disease are younger than five years.

"Children with Kawasaki disease who are not treated with high-dose intravenous gamma globulin (IVIG) within the first week to 10 days of fever onset have five times the risk of developing coronary artery aneurysms," Dr. Newburger says. "Sometimes the diagnosis of Kawasaki disease is not made until a child or adult (who had KD [Kawasaki disease] as a child) has a heart attack."

Although there are no laboratory diagnostic tests for Kawasaki disease, the classic criteria include fever for at least five days, plus four of the following five examination findings: ocular erythema typically without drainage or crusting; bright red lips and mouth and red, glossy "strawberry tongue;" and swollen, red, hands and feet followed by peeling of skin under the fingernails and toenails; rash predominantly in the groin area; and cervical lymphadenopathy with one node at least 1.5 cm.

For children in the acute phase of Kawasaki disease, the AHA still recommends treatment with IVIG and aspirin. Approximately 15% to 25% of untreated children develop coronary artery aneurysms or ectasia, but treatment with IVIG in the acute phase reduces this risk below 5%.

"The impact of Kawasaki disease in childhood on outcomes later in life depends to a large extent upon the amount of damage to the coronary arteries during the acute illness," Dr. Newburger says. "The new recommendations tailor the long-term management of the disease according to the threat to coronary health."

However, Dr. Newburger advises physicians to make treatment decisions based on each patient's individual condition.
Treatment with high-dose IVIG is recommended for children with fever of four days' duration and at least four of the five classic clinical criteria, as well as for those with fewer criteria but with coronary abnormalities noted on echocardiogram.

The new recommendations include an algorithm to assist clinicians in determining which children with fever for at least five days and fewer than four of the above criteria should be evaluated with an echocardiogram and/or receive IVIG treatment for Kawasaki disease.
IVIG retreatment may be useful for patients with persistent or recurrent fever after initial IVIG infusion. Other available treatments for children with IVIG-resistant Kawasaki disease include corticosteroids, tumor necrosis factor α antagonists, and abciximab.

Although about half of aneurysmal arterial segments resolve on angiography, these segments show persistent histologic and functional abnormalities. The remaining segments may continue to be aneurysmal and often develop progressive stenosis or occlusion.

The long-term management of patients with Kawasaki disease should be based on the degree of coronary involvement. Accordingly, the panel made specific recommendations for each risk level regarding antiplatelet and anticoagulant therapies, physical activity, follow-up assessment, and appropriate diagnostic tests to evaluate cardiac disease. However, the risk level for a given patient with coronary arterial involvement may change over time, reflecting changes in coronary artery morphology.

"Our statement on the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease is intended to provide practical interim recommendations until evidence based data are available to define best medical practices," the authors write.

Dr. Newburger has received research grants from Philips Medical and Pfizer and is a National Heart, Lung, and Blood Institute Advisory Council member. Coauthor Dr. Masato Takahashi reports receiving a research grant from Sanofi-Synthelabo, and Dr. Jane C. Burns received a research grant from Centocor.

Circulation. 2004;110:2747-2771