Abstract
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.
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Acknowledgements
This study was supported by the Ministry of Science and Technology, Taiwan (grant no. 107-2321-B-002 -026).
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Y.-C.L. and C.-H.C. worked for data collection and initial manuscript. F.-Y.K. and S.-K.H. provided statistics counseling. M.-H.W. worked for data interpretation. M.-T.L. had full access to all the data and takes responsibility for the integrity of the data and the accuracy of the data analysis.
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Liang, YC., Chang, CH., Lin, MT. et al. Shock and unresponsiveness to repeated courses of intravenous immunoglobulin in Kawasaki disease: a nationwide database study. Pediatr Res 87, 961–966 (2020). https://doi.org/10.1038/s41390-019-0668-1
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DOI: https://doi.org/10.1038/s41390-019-0668-1
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