الفهرس 
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Abstract
Systemic juvenile idiopathic arthritis; SJIA accounts for approximately 10 to 20% of all cases of JIA. It typically affects both sexes equally and may present in children as young as one year of age or younger. Approximately 10% of children with systemic JIA develop overt clinical features of macrophage activation syndrome (MAS), a life-threatening condition characterized by fever, organomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, hypo-fibrinogenemia, and coagulopathy and other findings.
The mortality rate for children hospitalized with systemic JIA and MAS is estimated to be as high as 6%, but may even be higher based on estimates from case series.
The main problem with MAS is the delayed diagnosis and the subsequent delayed introduction of proper therapy. MAS can overlap with sepsis making it difficult to be early controlled.
This study aims to evaluate measurement of the serum level of S100A4 in relation to disease activity and evolution of (MAS) in patients with systemic onset juvenile idiopathic arthritis (SJIA). The ultimate objective is to study the prognostic gain from adding this marker to the work up of this disease.
The study was conducted on 20 patients previously diagnosed as SJIA patients, three of them suffered secondary MAS, with mean age 9+3 years; followed up every 3 months over a whole year. Another group of 20 kids age and sex matched healthy controls.
Basic clinical evaluation and laboratory investigations were done. We found that SJIA activity patients had significantly higher levels of serum S100A4.
Multiple studied found that serum S100A4 is related to the disease pathogenesis, progress and chronicity in many autoimmune and autoinflmaatory diseases. However, this marker wasn’t studied before in relation to SJIA.
Serum S100A4 showed higher levels during activity than that during remission. This findings suggest that serum S100A4 increase with disease chronicity and surges up at time of activity.
We couldn’t assess the ability of S100A4 as predicator of MAS secondary to SJIA.
Serum S100A4 is positively correlated to serum ESR in SJIA patients.
We conclude that serum S100A4 is a useful marker for sJIA progression.
Even though, S100A4 needs wider-scale studies to value its measurement in pediatrics autoinflammatory diseases particularly sJIA, and to assess the efficacy of biological therapy on serum S100A4 expression in relation to disease progression and MAS development.