Journal of Pediatric Cardiology and Cardiac Surgery

It remains unclear how to treat patients with Kawasaki disease (KD) refractory to conventional intravenous gammaglobulin (IVIg) therapy. We report successful early aggressive combination treatment with ciclosporin and infliximab in an IVIg-resistant case. A 2-year 6-month-old boy with 5 principle symptoms was transferred to our hospital on day 3 of illness. Body temperature was 40.8°C, and appeared ill. Laboratory testing on admission showed: aspartate aminotransferase (AST), 2,439 IU/L; alanine aminotransferase (ALT), 1,142 IU/L; total bilirubin, 2.1 mg/dL; and C-reactive protein, 11.8 mg/dL. White blood cell count was 8.8×10⁹/L (85% neutrophils). Kobayashi, Egami, and Sano scores, each of which predict resistance to IVIg therapy, were 8, 5, and 3, respectively. Aspirin was not used as an anti-inflammatory agent, because of the elevated AST and ALT levels. IVIg and oral ciclosporin were administered on days 3 and 6, respectively, but fever persisted. After infliximab was administered on day 9, fever was immediately alleviated. He had no coronary aneurysms. This case suggested that third-line therapy should be performed by day 10 of illness to prevent coronary aneurysms. As additional treatment for IVIg-resistant KD, ciclosporin, infliximab and plasmapheresis should be considered, in that order. Further, aspirin may not be needed with aggressive therapies such as ciclosporin.