Prolonged Fever and Swollen Joints

  • Per Wekell
  • Stefan Berg
  • Anders Fasth


The diagnosis of systemic onset juvenile idiopathic arthritis (SoJIA) is based on clinical features including: prolonged quotidian fever, erythematous rash during fever spikes, generalized lymph node enlargement, arthralgia/arthritis and splenomegaly

Glucocorticoids are the first-line treatment of SoJIA

Approximately 10% of patients with SoJIA develop macrophage activation syndrome (MAS) associated with significant mortality

The main complication of SoJIA is MAS characterised by:

 Clinical features such as continuous fever, petechial rash, hepatosplenomegaly and compromised circulation,

 laboratory findings such as cytopenias in two or more cell lines, increased ferritin, liver transaminases, lactate dehydrogenase and triglycerides

There are no evidence-based treatment guidelines for MAS in SoJIA. Early expert advice should be sought. High-dose intravenous methylprednisolone is recommended as a first-line treatment


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Per Wekell
    • 1
    • 2
  • Stefan Berg
    • 2
    • 3
  • Anders Fasth
    • 2
    • 3
  1. 1.Department of PediatricsNU-Hospital GroupUddevallaSweden
  2. 2.Department of PediatricsSahlgrenska Academy, University of GothenburgGothenburgSweden
  3. 3.Department of Rheumatology and ImmunologyQueen Silvia Children’s HospitalGothenburgSweden

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