Kawasaki Disease or Incomplete Kawasaki Disease Clinical Pathway — Emergency Department and Inpatient


Recent Cochrane Review and meta-analysis have shed light on the utility of steroids in preventing coronary artery aneurysms. High-risk patients with KD benefit (decreased risk of coronary artery abnormality) from timely and potent adjunctive corticosteroid therapy. Longer courses of steroids, tapered over 2-3 weeks, appear superior to single doses of pulse steroids. Risk scoring systems used in Japan to determine high-risk status are not as reliable in North America. Young age is generally known to be a risk factor for worse outcomes. Elevated Z scores on initial echocardiogram of ≥ 2.0 demonstrated high predictive utility for later development of coronary artery abnormalities in one recent study in the US.

Most recent AHA guidelines note that administration of a longer course of steroids (tapering over 2-3 weeks) together with IVIG and ASA may be considered for treatment of high-risk patients when such high risk can be identified before initiation of treatment. These guidelines also state that administration of a longer course of steroids (tapering over 2-3 weeks) together with IVIG and ASA may be considered in the retreatment of patients with KD who have recurrent or recrudescent fever after initial IVIG treatment.

Chen st. al, Coronary Artery Complications in Kawasaki Disease and the Importance of Early Intervention, A Systematic Review and Meta-analysis, JAMA Pediatr. 2016;170(12):1156-1163

Wardle AJ, Connolly GM, Seager MJ, Tulloh RMR. Corticosteroids for the treatment of Kawasaki disease in children. Cochrane Database of Systematic Reviews 2017, Issue 1. Art. No.: CD011188. DOI: 10.1002/14651858.CD011188.pub2

Son. Et al Predicting Coronary Artery Aneurysms in Kawasaki Disease at a North American Center: An Assessment of Baseline Z scores. J Am Heart Assoc. 2017;6:e005378

  • Oral prednisolone/prednisone 2 mg/kg/day (max 60 mg/day) divided BID x 5 days then taper
  • OR
  • IV methylprednisolone 1 mg/kg/dose BID x 5 days then taper

  • Taper steroids over 2-3 weeks
  • Suggested taper (2mg/kg/day div BID x 5 days, 1 mg/kg/day div BID x 5 days, 0.5 mg/kg/day DAILY x 5 days)
Adverse Effects
  • Gastritis/Abdominal pain (consider famotidine while on steroids)
  • Hypertension
  • Bradycardia
  • Behavioral irritability, hallucination (consider morning dosing or re-timing medication to give earlier in day)