Abstract

Background: Although RAISE study demonstrated that intravenous immunoglobulins (IVIG) plus prednisolone (PSL) therapy for refractory Kawasaki disease (KD) reduced coronary artery aneurysm, the study population included a few incomplete KD. The aim of study was to evaluate the efficacy of RAISE protocol for incomplete KD. Methods: Children with incomplete KD which have 4 or less major symptoms of KD were enrolled. Using Kobayashi score, children were divided into low and high risk for IVIG resistance. Children with low risk group received IVIG monotherapy, whereas IVIG plus PSL therapy administrated for high risk group. Retrospectively, we assessed initial treatment response and coronary artery abnormalities (CAAs). Results: Overall, 63 incomplete KD (age; median and range 17 months (1-115 months), sex ratio; boys:girls 37:26) were enrolled. Median day of illness at diagnosis was 5 day of illness (2-10 days). Low risk group included 52 cases (83%) and the remaining 11 cases were high risk group. In low risk group, 87% of children (45 cases) were initial treatment responders. All 7 non-responders were responded to additional methylprednisolone steroid pulse therapy. All 11 children with high risk group were responders to the initial treatment. Five cases have equal to greater than 2.5 of Z-Score, which were all low risk group. All CAAs regressed to normal coronary diameter within 1 month. Conclusions: RAISE protocol was useful for the treatment of incomplete KD without any CAAs.

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