Still's disease

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Systemic-onset JIA (S-JIA) comprises around 10% of JIA.

  • it is responsible for a significant percentage of the morbidity and mortality caused by JIA
  • affects both female and males with the same frequency
  • may occur at any time during childhood

Presentation of the condition

  • arthritis
    • normally polyarticular
    • presents within the first 3 months of onset
  • extra-articular features
    • intermittent fever for at least 2 weeks plus one of the following
      • temperature rises up to 39.5 °C once or twice in a day
      • generally accompanied by a typical, salmon pink-coloured rash
    • typical rash
      • classic salmon-coloured evanescent rash
      • there is discrete circumscribed macules that may be surrounded by a ring of pallor or develop central clearing
      • vanishes with the decline of the fever
      • found most commonly on the trunk, axilla, and inguinal areas
      • may be exacerbated by stress, or a hot bath
    • generalized lymphadenopathy
      • seen in about one-third of patients
    • hepatosplenomegaly
      • seen in about one-third of patients
    • serositis
      • includes pericarditis and pleuritis

Note:

  • fever and rash generally resolve after the emergence of polyarthritis making the diagnosis of S-JIA difficult to differentiate from regular polyarticular JIA 

Reference:

Last reviewed 04/2021

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