title: Macrophage activation syndrome in Kawasaki disease: more common than we thought?
abstract: OBJECTIVE
  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.
authors.affiliation: The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Wang
authors.affiliation: The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China. Electronic address: gongfangqi@zju.edu.cn.
authors.forename: Fangqi
authors.initials: F
authors.lastname: Gong
authors.affiliation: The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China.
authors.forename: Weihua
authors.initials: W
authors.lastname: Zhu
authors.affiliation: The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China.
authors.forename: Songling
authors.initials: S
authors.lastname: Fu
authors.affiliation: The Children׳s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China.
authors.forename: Qing
authors.initials: Q
authors.lastname: Zhang
mesh_terms: D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003955:Diagnostic Tests, Routine / Q000379:methods* / Q000592:standards*; D005293:Ferritins / Q000097:blood; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D055501:Macrophage Activation Syndrome / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications*; D015995:Prevalence; D012680:Sensitivity and Specificity
chemical_list: D015415:Biomarkers; D005293:Ferritins; D007770:L-Lactate Dehydrogenase; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase
keywords: Kawasaki Disease; Macrophage activation syndrome