Kawasaki Disease

Pathway Background and Objective(s)

Kawasaki Disease is one of the most common vasculitides of childhood, and is the most common cause of acquired heart disease in children in developed countries. There is an estimated annual incidence of 20 per 100,000 children younger than five years in the United States, and prevalence is higher in Japan and in children of Japanese or East Asian descent. While it is typically a self-limited condition, complications such as coronary artery aneurysms, myocardial dysfunction, and heart failure may develop and lead to significant morbidity and mortality. The underlying etiology is unknown, and given the high risk of delayed diagnosis and/or treatment, it is imperative to standardize care to expedite recognition and timely treatment of Kawasaki Disease.

The objectives of this pathway are to:

• Standardize care of patients with Kawasaki Disease and Incomplete Kawasaki Disease
• Reduce the incidence of coronary artery aneurysms
• Reduce the time to IVIG treatment
• Reduce inpatient length of stay
• If steroids are used, reduce the incidence of refractory Kawasaki Disease

Algorithm

Download Kawasaki Disease Pathway Algorithm Updated September 23, 2021

Quality Metrics

• Percentage of patients with pathway order set usage
• Average time from admission to time of IVIG administration
• Number of patients with coronary artery aneurysms or ectasia at diagnosis
• Percentage of patients receiving medium dose aspirin in the acute phase of treatment
• Number of patients receiving steroids upon initial diagnosis
• Percentage of patients scheduled at discharge for follow up with a cardiologist
• Average length of stay (days)
• Number of patients readmitted due to Kawasaki Disease within 30 days

Educational Module

Download Kawasaki Disease Educational Module

Key References

• Abrams JY, Belay ED, Uehara R, Maddox RA, Schonberger LB, Makamura Y. Cardiac Complications, Earlier Treatment, and Initial Disease Severity in Kawasaki Disease. J Pediatr. 2017 Sept;188:64-69.
• Baker AL, Lu M, Minich LL, et al. Associated symptoms in the ten days before diagnosis of Kawasaki disease. J Pediatr. 2009 Apr; 154(4):592-595.
• Chen S, Dong Y, Kiuchi MG, et al. Coronary Artery Complication in Kawasaki Disease and the Importance of Early Intervention: A Systematic Review and Meta-analysis. JAMA Pediatr. 2016 Dec;170(12):1156-1163.
• McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. 2017 Apr;135(17):e927-e999.

Pathway Contacts

Marta Neubauer, MD (Pediatric Hospital Medicine)
Abby Theriaque, APRN (Pediatric Hospital Medicine)
Melissa Held, MD (Infectious Disease)
Lawrence Zemel, MD (Rheumatology)
Alex Golden, MD (Cardiology)

Disclaimer

The clinical pathways in the above links have been developed specifically for use at Connecticut Children’s and are made available publicly for informational and/or educational purposes only. The clinical pathways are not intended to be, nor are they, a substitute for individualized professional medical judgment, advice, diagnosis, or treatment. Although Connecticut Children’s makes all efforts to ensure the accuracy of the posted content, Connecticut Children’s makes no warranty of any kind as to the accuracy or completeness of the information or its fitness for use at any particular facility or in any individual case.

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