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Systemic Juvenile Idiopathic Arthritis Characteristics and Presentation

Solar-system Mobile Child with Idiot-hat King-Arthur
JulianMagome9668
Systemic Juvenile Arthritis (sJIA) is an idiopathic articular disease that is also known as Still's disease. Autoimmune destruction of joints is characteristic of this disease, and the onset is usually before age 16. This disease presents with polyarthritis, quotidian fever, an evanescent exanthem, and lymphadenopathy. Internal organs are also often involved, with hepatosplenomegaly being the most common manifestation.
9 KEY FACTS
CHARACTERISTICS
Idiopathic
Idiot-hat

Idiopathic means that the etiology is uncertain. Infections, stress, trauma and autoimmune origins are associated with sJIA.

Still's Disease
Whiskey Still

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
Auto-in-moon Joint with Exploding-destruction

Autoimmune joint destruction is implicated in the pathophysiology of sJIA.

Onset < 16 Years Old
On-switch Less-than Sweet (16) birthday cake

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.

PRESENTATION
Arthritis
King-Arthur

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 Fever
Quotation mark, Fever-beaver

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.

Evanescent Exanthem
Evanescent Evaporating in smoke, Exanthem "X" on skin

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
Lymph-lime-add (+)

Lymphadenopathy occurs alongside the quotidian fever and evanescent exanthem.

Hepatosplenomegaly
Liver-and-spleen-balloons

In sJIA, internal organs are involved to varying extents. Patients may experience hepatosplenomegaly, perimyocarditis, polyserositis, with pleuritis, and peritonitis.

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