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Macrophage activation syndrome in Kawasaki disease: more common than we thought?
Wei Wang1, Fangqi Gong1, Weihua Zhu1
1The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China.
Insights
Macrophage activation syndrome (MAS) is an under-recognized complication in Kawasaki Disease (KD). Current diagnostic criteria, like HLH 2009, show low sensitivity for MAS in KD patients.
Area of Science:
- Pediatrics
- Rheumatology
- Hematology
Background:
- Kawasaki Disease (KD) is a critical pediatric illness.
- Macrophage Activation Syndrome (MAS) is a severe complication that can arise in KD.
- Accurate diagnosis and management of MAS in KD are crucial for patient outcomes.
Purpose of the Study:
- To investigate the clinical features, treatment strategies, and outcomes of Kawasaki Disease patients with Macrophage Activation Syndrome.
- To compare the diagnostic performance of the HLH 2009 criteria versus Ravelli's criteria for MAS complicating KD.
Main Methods:
- Retrospective analysis of 719 Kawasaki Disease patients treated between 2007-2010.
- Evaluation of clinical and laboratory data to identify MAS.
- Comparison of diagnostic criteria (HLH 2009 and Ravelli's).
Main Results:
- Eight KD patients (1.11%) met Ravelli's criteria for MAS; only three (0.42%) met HLH 2009 criteria.
- Common laboratory abnormalities included elevated liver enzymes, ferritin, and LDH; cytopenia and hypertriglyceridemia were also frequent.
- Two cases developed coronary artery ectasia; seven were refractory to IVIG treatment.
Conclusions:
- MAS is likely an under-diagnosed complication of KD due to evolving diagnostic understanding.
- The HLH 2009 criteria demonstrate limited sensitivity and specificity for diagnosing MAS in KD.
- Consider MAS in KD patients with hepatosplenomegaly and specific laboratory findings (cytopenia, liver dysfunction, hyperferritinemia, elevated LDH, hypofibrinogenemia, hypertriglyceridemia).
Objectives:
To analyze the clinical characteristics, treatment, and outcomes of Kawasaki Disease (KD) patients associated with macrophage activation syndrome (MAS) and to compare two diagnostic standards (the HLH 2009 and Ravelli׳s criteria).
Methods:
All of the studied patients with Kawasaki Disease (KD) were treated at The Children׳s Hospital, Zhejiang University School of Medicine, during 2007-2010. Clinical and laboratory findings were analyzed.
Results:
In 719 KD patients, eight patients (1.11%, 81.3 ± 49.4 months, all male) were diagnosed by Ravelli׳s criteria, but only three (0.42%) patients were diagnosed by the HLH 2009 criteria. Aspartate aminotransferase increased significantly in all cases. Alanine aminotransferase, lactate dehydrogenase, and serum ferritin increased significantly in seven cases. Cytopenia and hypertriglyceridemia (>1.5mmol/L) were found in six and five cases, respectively. Hypofibrinogenemia (<1.5g/L) was found in two cases. Three cases showed evidence of hemophagocytosis, but only one case met the HLH 2009 criteria. Ectasia of the coronary arteries occurred in two cases. Seven patients were non-responsive to IVIG. One case died after the combined application of DXM, VP16, and CSA.
Conclusions:
MAS may be a frequently under-recognized complication of KD, because the understanding of complications and diagnostic criteria are still in progress. The HLH 2009 criteria have low sensitivity and specificity for the diagnosis of MAS complicating KD. When hepatosplenomegaly is present in KD patients with abnormal laboratory findings, such as cytopenia, liver dysfunction, hyperferritinemia, elevated serum LDH, hypofibrinogenemia, and hypertriglyceridemia, the presence of MAS should be considered.
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