Kawasaki disease (KD) is an acute
vasculitis of childhood that affects the medium vessels with a special predilection to the involvement of coronary arteries. The major morbidity of this disease is due to coronary artery
aneurysm, which occurs in about 25-30% of untreated cases. For decades now,
intravenous immunoglobulin (
IVIg) has consistently been shown to reduce the risk of CAAs to less than 5%. However, the mechanism of
immunomodulation remains unclear. Several studies on the role of
IVIg in the modulation of
toll-like receptor pathways, autophagy, and apoptosis of the mononuclear phagocytic system, neutrophil extracellular trap, and dendritic cell modulation suggest a modulatory effect on the innate immune system. Similarly, certain studies have shown its effect on T-cell differentiation,
cytokine release, and regulatory T-cell function. In this review, we discuss the potential mechanisms underlying the immunomodulatory actions of
IVIg in patients with
Kawasaki disease. Furthermore, we provide a summary of the evidence regarding various infusion protocols and dosages utilized in the treatment of KD patients.