Idiopathic means that the etiology is uncertain. Infections, stress, trauma and autoimmune origins are associated with sJIA.
Systemic juvenile idiopathic arthritis was previously known as Still's disease, after Sir George Frederic Still, an English physician. Adult-onset Still's disease occurs after age 16.
Autoimmune joint destruction is implicated in the pathophysiology of sJIA.
sJIA occurs in toddlers, possibly as early as the 1st year of life and peaks in the 2nd – 5th year of life. Onset in school children is rare. Boys and girls get sick equally often. Systemic onset juvenile arthritis represents about 10% of juvenile idiopathic arthritis cases.
Arthritis associated with sJIA usually manifests as a polyarthritis and rarely as an oligoarthritis. It can occur at the same time as the fever or weeks, months, and occasionally years after the initial fever.
Quotidian (Latin for "occurring daily") fever is very characteristic of this disease. It is a high, intermittent fever which often rises above 39° C in the morning and lasts for at least 2 weeks.
The fever is combined with a pale red, polymorphic, small-spotted rash. Often, this rash is only found during the fever and is associated with lymphadenopathy.
Lymphadenopathy occurs alongside the quotidian fever and evanescent exanthem.
In sJIA, internal organs are involved to varying extents. Patients may experience hepatosplenomegaly, perimyocarditis, polyserositis, with pleuritis, and peritonitis.
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