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High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant
  1. Marie Lind-Holst,
  2. Ulla Birgitte Hartling and
  3. Anne Estmann Christensen
  1. Hans Christian Andersen Children’s Hospital, Odense University Hospital, Odense, Denmark
  1. Correspondence to Dr Marie Lind-Holst, marie.lind-holst{at}


We report a 12-week-old boy presenting with incomplete refractory Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS). The infant presented with cerebral irritability, pain, tachypnoea and vomiting for 10 days. He did not fulfil any of the classic diagnostic criteria for KD. Pericardial effusion on echocardiography in addition to severe dilatation of the coronary arteries in combination with leucocytosis and raised acute phase reactants led to the diagnosis of incomplete KD. Treatment with intravenous immunoglobulin and aspirin was initiated but without any response. The condition was subsequently refractory to additional treatment with infliximab and high-dose methylprednisolone. His condition worsened, fulfilling the criteria for MAS. High-dose anakinra was initiated, and remission of the inflammation was achieved.

  • paediatrics (drugs and medicines)
  • vasculitis
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  • Contributors ML-H has been in charge of the writing process of this case report and made changes to the manuscript. She has made the figure regarding the laboratory findings and MAS criteria and the submission and resubmission. UBH supported with the information on genetic and immune deficiency investigation and contributed with grammar correction. AEC has been supportive with knowledge of expert level and supported with input on the focus of this case report. She was responsible for the correction of language and grammar.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Parental/guardian consent obtained.

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