ACR: Continue daily monitoring in children with Kawasaki disease who have resolved fevers
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PHILADELPHIA — In children with acute Kawasaki disease where fever symptoms have resolved, continued daily monitoring for 1 to 2 weeks is strongly recommended, according to new guidelines released during ACR Convergence 2022.
“[These are the] first guidelines on Kawasaki disease from the ACR and the Vasculitis Foundation,” Sangeeta Sule, MD, PhD, chief of the rheumatology division at Children’s National Hospital, in Washington, D.C., told attendees. “It is a vasculitis, which is why we want to address this early, to prevent complications such as coronary artery aneurisms.”
A voting panel consisting of adult and pediatric rheumatologists, pediatric cardiologists and infectious disease physicians, patient representatives and experts from the ACR and Vasculitis Foundation determined the new guidelines for the management of Kawasaki disease.
The literature review informing the guidelines was based on 16 questions addressing Kawasaki disease treatment and management, as well as diagnostic testing. The questions focused on fields including the population and problem, relevant interventions, alternative interventions and relevant outcomes for the patient population.
There are a total of 14 guidelines for the treatment Kawasaki disease. They are:
- Intravenous immunoglobin (IVIG) is considered the standard of care for the initial treatment course of Kawasaki disease. The recommended dose is 2 grams per kilogram.
- IVIG combined with glucocorticoid adjunctive therapy is conditionally recommended over IVIG alone.
- IVIG combined with nonglucocorticoid immunomodulatory therapy is conditionally recommended over IVIG alone.
- Repeat courses of IVIG are conditionally recommended over glucocorticoid therapy.
- As an ungraded position statement, glucocorticoid or nonglucocorticoid immunomodulatory therapy may be considered if there is a persistent fever following two doses of IVIG.
- Aspirin is strongly recommended and is considered standard of care.
Additionally, among patients with Kawasaki disease and macrophage activation syndrome, Kawasaki disease should be treated with IVIG as the first-line option. Meanwhile, in patients with macrophage activation syndrome, it is strongly recommended that patients be treated with therapies that target cytokine storms or underlying triggers.
In patients with acute Kawasaki disease whose fevers resolve, continued daily monitoring is strongly recommended for 1 to 2 weeks, while those with persistent arthritis following therapy with IVIG, can receive NSAIDs to treat symptoms.
For patients with incomplete Kawasaki disease, it is recommended to begin therapy at the time of diagnosis, and to retrieve echocardiogram with coronary artery measurements.
In children with shock physiology, it is strongly recommended to obtain an echocardiogram with coronary artery measurements. Lastly, in patients with unexplained macrophage activation syndrome, it is strongly recommended to obtain an echocardiogram with coronary artery measurements.
“The goal here was to develop guidelines based on recommendations and expertise comprised by the literature review team,” Sule said. “The guidelines are really focused on a U.S.-based audience, so what you will see here is directed toward the U.S. practitioner.”
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Sule S. 2021 ACR Kawasaki disease guideline. Presented at: ACR Convergence 2022; Nov. 11-15, 2022; Philadelphia (hybrid meeting).
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