• Kawasaki’s Disease (KD) is one of the most common causes of vasculitis and a leading cause of acquired heart disease in children.
• Boys are affected more commonly than girls.
• 80-90% of cases occur in patients under the age of 5.
• The underlying etiology is unknown.
• Its incidence is highest in Japan at 265 cases per 100,000 children under the age of 5. In the USA, incidence is 20 cases per 100,000 children under the age of 5.
• Usually a self-limiting condition characterized by a prolonged fever with other acute inflammatory manifestations lasting 12 days on average.
• 20-25% of untreated cases develop coronary artery aneurysms, which could lead to myocardial ischemia, infarction, or sudden death. Early recognition and treatment with intravenous immunoglobulin (IVIG) will lower this rate of aneurysms to less than 5%.
• Diagnosis = fever (typically above 38.5ºC) persisting > 5 days AND > 4 of:
  • Bilateral non-exudative conjunctival injection, usually limbic-sparing.
  • Oral mucous membrane changes (e.g. erythema and/or fissuring of lips, strawberry tongue, and/or erythema of oropharyngeal mucosa).
  • Peripheral extremity changes.
  • Acute phase: erythema of palms and/or soles and/or edema of hands and/or feet.
  • Subacute phase: periungual desquamation.
  • Polymorphous rash (maculopapular, diffuse erythroderma, or erythema multiforme-like).
  • Cervical lymphadenopathy (at least one lymph node ≥1.5 cm in diameter, usually unilateral).
•  Algorithm of evaluating suspected KD below:

created by Ella Barrett-Chan, MSI UBC

• Immediate treatment includes:
  • IVIG 2 grams/kg x 1 dose over 8 to 12 hours.
  • High-dose acetylsalicylic acid 30 to 50 mg/kg/day in 4 divided doses up to 4 grams per day until fever resolves, then give low-dose acetylsalicylic acid (local guidelines may differ).
  • Consider glucocorticoids 2 mg/kg/day PO or IV in three divided doses for 10 days then 1 mg/kg/day for 5 days if history of IVIG resistance noted (local guidelines may differ).
• Further investigations and treatments listed in algorithm below:

created by Ella Barrett-Chan, MSI UBC

All patients diagnosed with KD need referral and admission.

Dependent on local guidelines.

Suspicion of KD in a patient warrants consult to pediatrics/internal medicine and cardiology.

Dependent on patient’s clinical response to treatment and echocardiogram results.

1. Husain, N., & Carr, M. (2018). Coronary artery emergencies in children. Clinical Pediatric Emergency Medicine, 19(4), 367-372. https://doi.org/10.1016/j.cpem.2018.12.011.

   
   

2. McCrindle, B. W., Rowley, A. H., Newburger, J. W., Burns, J. C., Bolger, A. F., Gewitz, M., … & Kobayashi, T. (2017). Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation, 135(17), e927-e999.

   
   

3. Muzumdar, S., Rothe, M. K., & Grant-Kels, J. M. (2019). The rash with maculopapules and fever in children. Clinics in Dermatology, 37(2), 119-128.

   
   

4. Sundel, R. (2019). Kawasaki disease: Initial treatment and prognosis. In E. Tepas (Ed.), UpToDate. 

   
   

5. Yamamoto, L. G., & Martin, J. E. (1994). Kawasaki syndrome in the ED. The American Journal of Emergency Medicine, 12(2), 178-182.