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March 09, 2022
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ACR, Vasculitis Foundation guideline emphasizes prompt IVIG for Kawasaki disease

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The American College of Rheumatology and the Vasculitis Foundation have released new guidelines for Kawasaki disease, focusing on diagnostic issues, high-risk patients and convalescent patient management.

Kawasaki disease continues to be an area of evolving understanding in clinical treatment,” said Mark Gorelik, MD, an assistant professor at Columbia University Vagelos College of Physicians and Surgeons in New York, and the lead investigator of the guideline, said in a press release. “These guidelines will help clinicians better treat patients by augmenting existing guidelines from the American Heart Association, especially for complex patients seen by rheumatologists.”

“IVIG is the central therapy for patients with Kawasaki disease and should be administered as soon as the diagnosis of Kawasaki disease is made,” Mark Gorelik, MD, said in the release. Source: Adobe Stock

In total, the new guidelines include 11 treatment recommendations, a good practice statement that “all Kawasaki disease patients should be initially treated with intravenous immunoglobulin (IVIG),” and an ungraded position statement on the use of “non-glucocorticoid immunosuppressive therapy or glucocorticoids for patients with acute Kawasaki disease and persistent fevers after repeated treatment with IVIG.”

Strong recommendations include early treatment of incomplete Kawasaki disease, aspirin treatment and gathering an echocardiogram when there are cases of shock, according to the release. Conditional recommendations include using IVIG in conjunction with other therapies for patients with “high-risk features IVIG resistance and/or coronary artery aneurysms.”

“IVIG is the central therapy for patients with Kawasaki disease and should be administered as soon as the diagnosis of Kawasaki disease is made,” Gorelik said in the release.

The guidelines also include two differences from current standards. The first is that higher-risk patients should be treated with short courses of corticosteroids when diagnosed. The second is that either high- or low-dose aspirin may be used for therapy, according to the release.

The paper detailing the new recommendations in whole is available on the ACR website.