Initial treatment for Kawasaki disease based upon risk factors for IVIG resistance

KD: Kawasaki disease; CA: coronary artery; IVIG: intravenous immune globulin; ESR: erythrocyte sedimentation rate; IV: intravenous; IM: intramuscular.
* The initial echocardiogram is typically performed on presentation prior to IVIG treatment. However, IVIG treatment should not be delayed if the echocardiogram cannot be performed quickly. If enlarged CAs are noted on an initial echocardiogram performed after the first dose of IVIG is administered, the patient should still receive additional treatment with glucocorticoids.
¶ Patients in this category are at high risk for development of CA aneurysms and should be followed more carefully, including earlier and more frequent clinical, laboratory, and echocardiographic evaluations. Patients who are initially determined not to be at high risk but who do not respond to treatment with a single dose of IVIG without glucocorticoids are also at increased risk for development of CA aneurysms and should be followed in a similar manner.
Δ IVIG should be administered in a health care setting with equipment, medications (eg, IM epinephrine), and staff who are trained to respond in the event of a rare serious reaction (eg, anaphylaxis). Patients should be well hydrated prior to IVIG administration. Infusion rate is adjusted based on tolerability. Some patients may receive acetaminophen and/or H1 antihistamine prior to or during the infusion to prevent or blunt an infusion reaction. For specific recommendations, refer to UpToDate topics on overview of IVIG therapy and IVIG adverse effects.
◊ Normalization of ESR typically takes 1 to 2 months. Thus, aspirin therapy is generally complete within 2 months of disease onset in children with no CA abnormalities.
§ IV prednisolone may be unavailable in some settings (eg, United States). The equivalent dose of methylprednisolone is 1.6 mg/kg/day for 10 days then 0.8 mg/kg/day for 5 days. A single IV dose of infliximab (5 mg/kg) is an alternative to glucocorticoids. For additional detail, including considerations in the timing of switch from IV to oral glucocorticoid, refer to UpToDate topic on treatment of KD.