From the Guidelines
Intravenous Immunoglobulin (IVIG) is the primary treatment for Kawasaki disease and should be administered as soon as the diagnosis is made, with a recommended dose of 2 g/kg given as a single infusion over 10-12 hours, as supported by the most recent and highest quality study 1.
Key Points
- IVIG is most effective when given within the first 10 days of illness, ideally within 7 days, to reduce the risk of coronary artery abnormalities.
- It should be administered along with high-dose aspirin (80-100 mg/kg/day divided into four doses) during the acute phase.
- IVIG works by modulating the inflammatory response, neutralizing bacterial superantigens, providing anti-cytokine effects, and suppressing endothelial cell activation.
- This helps reduce fever, resolve clinical symptoms, and most importantly, decreases the incidence of coronary artery aneurysms from approximately 25% to less than 5% 1.
Additional Considerations
- About 10-20% of patients may be resistant to initial IVIG therapy and require a second dose or additional treatments.
- Potential side effects include headache, myalgia, fever, and rarely, aseptic meningitis or anaphylaxis, so patients should be monitored during infusion.
- For children with IVIG resistance, a second IVIG dose, corticosteroids, or biologics like infliximab may be considered as additional therapy, as suggested by studies 1.
Mechanism of Action
- The mechanism of action of IVIG in treating Kawasaki disease is unknown, but it appears to have a generalized anti-inflammatory effect, with possible mechanisms including modulation of cytokine production, neutralization of toxins or other pathogenic agents, augmentation of regulatory T-cell activity, suppression of antibody synthesis, and provision of anti-idiotypic antibodies 1.
From the Research
Role of IVIG in Treating Kawasaki Disease
- IVIG is used to treat Kawasaki disease due to its ability to reduce the incidence of coronary artery abnormalities (CAAs) 2, 3.
- The primary treatment for Kawasaki disease is IVIG and acetylsalicylic acid (ASA), with doses and regimens differing between institutions 2.
- High-dose IVIG regimens are probably associated with a reduced risk of CAA formation compared to ASA or medium- or low-dose IVIG regimens 2.
- IVIG probably reduces the incidence of CAAs in people with Kawasaki disease up to 30 days, and may also reduce the duration of fever 2.
Comparison of IVIG with Other Treatments
- Compared to ASA, high-dose IVIG probably reduces the duration of fever, but there is little or no difference detected in the need for additional treatment 2.
- Combination of corticosteroid with IVIG as an initial treatment strategy could reduce the risk of coronary abnormality 4.
- IVIG alone may be non-inferior to IVIG combined with high-dose aspirin for reducing coronary artery lesions (CALs) in children with Kawasaki disease 5.
- Low-dose ASA was associated with higher odds of IVIG resistance compared to high-dose ASA, with no significant difference in duration of hospital stay or incidence of coronary artery aneurysms 6.
Efficacy and Safety of IVIG
- The available evidence indicates that high-dose IVIG regimens are probably associated with a reduced risk of CAA formation compared to ASA or medium- or low-dose IVIG regimens 2.
- There were no clinically significant differences in incidence of adverse effects between IVIG and other treatment groups, suggesting little concern about the safety of IVIG 2, 4.
- The certainty of the evidence ranged from moderate to very low due to concerns related to risk of bias, imprecision, and inconsistency 2.