Shock and unresponsiveness to repeated courses of intravenous immunoglobulin in Kawasaki disease: a nationwide database study

Background We aimed to investigate the clinical implications of unresponsiveness to single or repeated courses intravenous immunoglobulin (IVIG) and Kawasaki disease (KD) shock syndrome in patients with KD in an era of a single brand of IVIG. Methods Data were collected from National Health Insurance database 2010-2013. Characteristics of the KD patients were analyzed, including age, gender, shock, and associated coronary aneurysms. Results There were 3043 KD patients (male: 1872) identified. Among them, 46 (1.51%) had KDSS, 261 patients (8.5%) had IVIG unresponsiveness, and 225 patients (7.4%) developed coronary aneurysms. Moreover, 51 patients did not respond to the second course IVIG therapy, i.e., re-IVIG unresponsiveness. KDSS was associated with the occurrence of IVIG unresponsiveness (P < 10(-4)) and re-IVIG unresponsiveness (P = 0.02). In addition to male gender and KD shock syndrome, IVIG unresponsiveness (OR: 2.18, 95% CI: 1.48-3.22, P = 0.001) and re-IVIG unresponsiveness (OR: 2.87, 95% CI: 1.40-5.89, P = 0.004) were both independent risk factors for coronary aneurysms. Conclusions In a nationwide KD cohort, both IVIG unresponsiveness and re-IVIG unresponsiveness increase the risk of coronary aneurysms. Such observation addresses the importance of refining the treatment for IVIG unresponsiveness, at least in those with KD shock syndrome.