On-line First

J Rheum Dis

Published online February 3, 2025

© Korean College of Rheumatology

Macrophage activation syndrome in Kawasaki disease: a literature review of Korean studies

Dae Chul Jeong, M.D., Ph.D.1,2 , Soo-Young Lee, M.D. Ph.D.1,2

1Department of Pediatrics, College of Medicine, The Catholic University of Korea, 2The Vaccine Bio Research Institute, College of Medicine, The Catholic University of Korea, Seoul, Korea

Correspondence to : Soo-Young Lee, https://orcid.org/0000-0002-5354-3135
Department of Pediatrics, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 327 Sosaro, Wonmi-gu, Bucheon 14647, Korea. E-mail: sylee@catholic.ac.kr

Received: October 17, 2024; Revised: December 18, 2024; Accepted: January 15, 2025

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Macrophage activation syndrome (MAS) is a rare but potentially life-threatening complication of Kawasaki disease (KD). In Korea, many studies on KD have been reported, but there are only a few studies on MAS complicating KD (MAS-KD). This study was conducted to provide the characteristics of MAS-KD patients in Korea through a literature review. A total of 23 Korean patients with MAS-KD from 10 papers were included in this study. All MAS-KD patients met the hemophagocytic lymphohistiocytosis (HLH)-2004 criteria and/or the 2016 MAS criteria. The incidence of MAS-KD in Korean children was 0.8%~1.1%, which is relatively low compared to North America (1.9%). MAS-KD patients had lower rates of KD-related features and higher rates of incomplete KD, coronary artery abnormalities, and intravenous immunoglobulin resistance than patients with KD without MAS. Notable laboratory abnormalities in MAS-KD include anemia, neutropenia, thrombocytopenia, hypoalbuminemia, increased hepatic transaminase levels, and hyperferritinemia. For treatment of MAS-KD, the HLH-2004 protocol (i.e., 40 weeks of complex chemotherapy) was applied to 15 patients (65%), which is a significantly greater than those treated with this protocol in other countries (35%). Two patients (9%) died during the HLH-2004 protocol. In actual practice, MAS may be underrecognized in patients with KD. Clinical suspicion is paramount for early diagnosis and timely treatment.

Keywords Macrophage activation syndrome, Kawasaki disease, Korea, Child

Article

On-line First

J Rheum Dis

Published online February 3, 2025

Copyright © Korean College of Rheumatology.

Macrophage activation syndrome in Kawasaki disease: a literature review of Korean studies

Dae Chul Jeong, M.D., Ph.D.1,2 , Soo-Young Lee, M.D. Ph.D.1,2

1Department of Pediatrics, College of Medicine, The Catholic University of Korea, 2The Vaccine Bio Research Institute, College of Medicine, The Catholic University of Korea, Seoul, Korea

Correspondence to:Soo-Young Lee, https://orcid.org/0000-0002-5354-3135
Department of Pediatrics, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 327 Sosaro, Wonmi-gu, Bucheon 14647, Korea. E-mail: sylee@catholic.ac.kr

Received: October 17, 2024; Revised: December 18, 2024; Accepted: January 15, 2025

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Macrophage activation syndrome (MAS) is a rare but potentially life-threatening complication of Kawasaki disease (KD). In Korea, many studies on KD have been reported, but there are only a few studies on MAS complicating KD (MAS-KD). This study was conducted to provide the characteristics of MAS-KD patients in Korea through a literature review. A total of 23 Korean patients with MAS-KD from 10 papers were included in this study. All MAS-KD patients met the hemophagocytic lymphohistiocytosis (HLH)-2004 criteria and/or the 2016 MAS criteria. The incidence of MAS-KD in Korean children was 0.8%~1.1%, which is relatively low compared to North America (1.9%). MAS-KD patients had lower rates of KD-related features and higher rates of incomplete KD, coronary artery abnormalities, and intravenous immunoglobulin resistance than patients with KD without MAS. Notable laboratory abnormalities in MAS-KD include anemia, neutropenia, thrombocytopenia, hypoalbuminemia, increased hepatic transaminase levels, and hyperferritinemia. For treatment of MAS-KD, the HLH-2004 protocol (i.e., 40 weeks of complex chemotherapy) was applied to 15 patients (65%), which is a significantly greater than those treated with this protocol in other countries (35%). Two patients (9%) died during the HLH-2004 protocol. In actual practice, MAS may be underrecognized in patients with KD. Clinical suspicion is paramount for early diagnosis and timely treatment.

Keywords: Macrophage activation syndrome, Kawasaki disease, Korea, Child

JRD
Jan 01, 2025 Vol.32 No.1, pp. 1~7
COVER PICTURE
Cumulative growth of rheumatology members and specialists (1980~2024). Cumulative distribution of the number of the (A) Korean College of Rheumatology members and (B) rheumatology specialists. (J Rheum Dis 2025;32:63-65)

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