Still's disease
Last reviewed 04/2021
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
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