systemic Juvenile Idiopathic Arthritis
Also classified as
sJIA, Still’s disease
Research – Resources – Information
Clinical Characteristics of sJIA
sJIA represents 10–15% of all JIA, with a broad peak of onset between 0 and 5 years of age, with 2 years being the most common , and an equal sex ratio. It is called Still’s disease (AoSD) when it occurs in patients over the age of 16. AoSD is less common than sJIA but the disease features are the same, even severe arthritis occurs exceptionally. Therefore, sJIA and AoSD likely represent a continuum of the same disease entity .
SJIA is defined by  the presence of arthritis in one or more joints associated with spiking fever (a typically daily high fever with spike in the evening) persisting for a minimum of 15 days, with at least one of the following manifestations: skin rash (evanescent, non-fixed erythematous rash that accompanies fever spikes), generalized lymphadenopathy, hepatomegaly and/or splenomegaly, or serositis (pleuritis or pericarditis).
None of the clinical signs is specific to sJIA, especially at presentation, and differential diagnosis can be difficult (bacterial and viral infections, malignancy, and other rheumatic diseases). Moreover, arthritis may be absent at onset and can develop during disease course, usually progressing to a polyarticular and symmetrical involvement.
The disease course can be highly variable. It can be monocyclic, polycyclic with relapses followed by intervals of remission, or unremitting, leading about half of the patients to a chronic destructive arthritis representing the major long-term problem.
SJIA shows a strong association with macrophage activation syndrome (MAS), a form of reactive hemophagocytic lymphohistiocytosis (HLH), characterised by an uncontrolled activation of well-differentiated macrophages releasing a high amount of proinflammatory cytokines, particularly IL-18, which belongs to the IL-1 family. MAS is a severe, potentially life-threatening disorder, and clinically characterized by fever, hepatosplenomegaly, lymphadenopathy, neurologic dysfunction, and coagulopathy. Some studies suggest that up to 50% of sJIA patients might have occult MAS [7, 8]. Heterozygous mutations in genes involved in HLH have been described in some subsets of SoJIA patients and might play a role in the development of MAS . Specific criteria for sJIA-associated MAS have been recently proposed . Interestingly, MAS has been recently included as an individual group of autoinflammatory diseases in an updated classification proposed by Masters et al. .
Taken from the International Journal of Inflammation
Volume 2012 (2012), Article ID 480373, 5 pages
BROCHURE WRITTEN AND COMPILED
TWO sJIA PATIENTS
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Donated to the International Still’s Disease Foundation, Inc.
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