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Prolonged Fever, Rash and Mucosal Bleeding

  • Beata DerfalviEmail author
Chapter

Abstract

  • Macrophage activation syndrome (MAS) presents at onset in up to 22% of patients and in up to 50% in subclinical forms of systemic juvenile idiopathic arthritis (SJIA). About 10–15% of children with SJIA experinece at least one episode of MAS during their disease

  • It is very difficult to distinguish SJIA/MAS from primary HLH, especially in a very young child

  • An initially high ESR that then drops, despite increasing inflammation, is characteristic of MAS/HLH and thought to be secondary to decreasing fibrinogen level

  • Currently there are no validated evidence-based guidelines on management of MAS in SJIA

  • A short-term, high-dose intravenous methylprednisolone therapy is reported to be an effective first-line treatment

Keywords

Systemic juvenile idiopathic arthritis Macrophage activation syndrome Triglyceride Ferritin 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of PediatricsDalhousie University/IWK Health CentreHalifaxCanada

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