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

Steroids

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

Dose	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)

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

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