STRATEGY FOR HIGH-DOSE IMMUNOGLOBULIN THERAPY–RESISTANT KAWASAKI DISEASE: CURRENT STATUS IN JAPAN

Abstract

INTRODUCTION: High-dose intravenous immunoglobulin (IVIg) therapy has decreased the risk for development of coronary arterial lesions (CALs) in Kawasaki disease (KD), whereas patients who show resistance to IVIg have a higher risk for CALs. OBJECTIVE: The purpose of this study was to determine the risk for CALs in patients with IVIg-resistant KD and to investigate whether an additional therapy might affect its risk, based on the nationwide survey (2003–2004) in Japan. METHODS: Information from 11 510 children with KD treated with IVIg with the first 9 days of illness was available. The incidence of CALs was compared among 4 groups: group 1 (G1), children who responded to initial IVIg; group 2 (G2), IVIg-resistant patients who received additional IVIg; group 3 (G3), IVIg-resistant patients who received additional prednisolone (PSL); and group 4 (G4), IVIg-resistant patients who received additional IVIg plus PSL. CALs were assessed on the 30th day of illness. RESULTS: Among 11 510 cases, 2229 patients (19.4%) were resistant to initial IVIg treatment and received additional therapy. The incidence of CALs was significantly lower in children who responded to IVIg (G1, n = 9281) than in those with IVIg resistance (1.87% and 11.03%, respectively). In each of the additional therapy groups, the incidences of CALs were as follows: G2 (n = 1108), 6.68%; G3 (n = 93), 9.68%; and G4 (n = 135), 22.22%. Thus, the risk for development of CALs was significantly higher for patients in G4 than those in G1 and G2. CONCLUSIONS: Additional therapy including PSL may increase the risk for CALs; however, several selection biases, such as more severe cases in G3 and G4, might have affected the results.