Abstract

Backgrounds: In Japan, infliximab (IFX) has been used in patients with Kawasaki disease (KD) who did not respond to initial treatment. However, nationwide surveillance of the IFX for KD patients has not been reported. The objective of the present study is to reveal epidemiological and clinical features of KD patients treated with IFX. Method: This retrospective observational study was conducted from 2007 to 2013. We sent a questionnaire to members of Japanese Society of Kawasaki disease annually and collected demographic data and clinical outcomes of KD patients treated with IFX. A total of 315 patients (208 boys and 107 girls, age at onset 37±25 months, illness days of initial treatment 4.3±1.2) from 46 hospitals were identified and analyzed in the present study. Results: No patients received IFX as initial treatment. Over 60% of the patients were treated with IFX as first or second line additional rescue treatment. After the IFX administration, 237 patients (75.7%) became afebrile within 24 hours and 82 patients (26.0%) treated additional rescue treatments subsequently. Before IFX administration, 55 patients already had coronary artery aneurysm exceeding 4mm and 69 patients were finally affected coronary artery aneurysm exceeding 4mm. Serum level of C reactive protein were significantly decreased after IFX administration (10.6±7.2 to 4.9±4.7, P<0.001). Patients who required additional treatment after IFX showed significantly higher CRP levels before IFX compared to patients without additional treatment after IFX (12.3±8.3 vs. 10.0±6.7, P=0.049). Adverse events were observed in 50 patients (15.9%) which recovered spontaneously or after interventions. Conclusions: IFX therapy for Kawasaki disease patients who did not respond to the initial treatment would be well tolerated and play a certain role to improve clinical course.

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