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

CA: coronary artery; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; IM: intramuscular; IV: intravenous; IVIG: intravenous immune globulin; KD: Kawasaki disease; LAD: left anterior descending artery; RCA: right coronary artery.

* 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). 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.