What is the role of IV immunoglobulin (IVIG) in the treatment of Kawasaki disease?

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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IVIG relieves acute inflammation and has been shown to reduce the rate of CAAs from approximately 25% in untreated patients to 3-5% in treated patients. [2]  Maximal benefits are seen when IVIG is given within the first 10 days after the onset of fever. Some controversy exists about the ideal time to begin IVIG, but it is given most often from days 5-7.

In the past, IVIG was given as a lower dose over 4 days (400 mg/kg/day), but newer studies have shown that single high doses are more effective. In current practice, the dose is 2 g/kg intravenously over 10-12 hours. [9]

10-15% of patients will fail initial treatment with IVIG; treatment failure is defined as a fever occurring 36 hours or longer after IVIG is administered. In many of these cases, a second treatment with IVIG at the original dose is recommended. [69] A small subgroup of patients fails to respond to a second dose of IVIG (see Treatment/Treatment of IVIG-Resistant Disease).

A study in an ethnically diverse population in San Diego, California, found that patients with IVIG resistance tended to have higher percent bands, CRP, alanine aminotransferase, and gamma-glutamyl transferase, as well as lower platelet counts and age-adjusted hemoglobin concentrations. They were also more likely to have CAAs. However, a proposed scoring system to predict IVIG resistance proved insufficiently accurate to be clinically useful. [70]

In a review from Singapore by Sittiwangkul et al, initial treatment with IVIG (2 g/kg) failed to elicit a response in 13% of patients. [71] The diagnosis in 2 patients with IVIG-resistant KD was delayed, and giant aneurysms developed. Patients with a high ESR were at an increased risk of IVIG-resistant Kawasaki disease. Patients with IVIG-resistant KD had a higher prevalence of coronary artery lesions at the acute phase and 2 months after onset. [71]

It is important to note that the American Academy of Pediatrics Red Book states that live vaccinations are contraindicated for 11 months after administration of IVIG for KD.

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