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Front. Pediatr. | doi: 10.3389/fped.2019.00423

Kawasaki Disease Complicated with Macrophage Activation Syndrome: Case Reports and Literature Review

 Ping Jin1,  Yong Luo1, Xi Liu1, Jin J. Xu1 and  Chun Y. Liu1*
  • 1Baoan Maternal and Child Health Hospital, Jinan University, China

Kawasaki disease (KD) is among the most common forms of vasculitis in children. Macrophage activation syndrome (MAS) is a rare and severe complication of KD. In this study, we retrospectively analyzed three KD patients complicated with MAS who were treated in pediatric intensive care units (PICU) and reviewed the relevant literature. We reported that being male, being over 2 years of age, incomplete KD, intravenous immunoglobulin (IVIG) resistance, or persistent fever for more than 10 days are all highly associated with MAS. Relevant examinations should be performed as early as possible to detect MAS. Patients with KD complicated with MAS are at a high risk of having coronary artery involvement or aneurysm formation, which can be reversed with timely treatment. For KD patients with IVIG resistance, additional treatments should be administrated, including a second dose of IVIG (2 g/kg), glucocorticoids, tumor necrosis factor (TNF) inhibitors, and other immunosuppressive drugs or plasma exchange. Early identification and timely treatment are key points for improving the prognosis of KD patients complicated with MAS.

Keywords: kawasaki disease, complication, Severe, Macrophage Activation Syndrome, Children

Received: 30 Jun 2019; Accepted: 04 Oct 2019.

Copyright: © 2019 Jin, Luo, Liu, Xu and Liu. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. Chun Y. Liu, Baoan Maternal and Child Health Hospital, Jinan University, Shenzhen, China, chunyi618@163.com