الفهرس 
? Systemic Juvenile Idiopathic ArthritisOnly 14 pages are availabe for public view
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Abstract
Objective: This study aims to evaluate measurement of the serum level of survivin as an anti-apoptosis marker in relation to disease activity and evolution of macrophage activation syndrome (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. Participants and methods: study is a pilot prospective study, conducted in the Pediatric Allergy and Immunology section, Children’s Hospital, Ain Shams University. Twenty two SJIA patients who were diagnosed on basis of American College of Rheumatology (ACR) for diagnosis of SJIA, as well as the International League of Association for Rheumatology (ILAR) classification of juvenile idiopathic arthritis, with mean age 10+- 3 years; followed up every 2 months over a whole year. Basic clinical evaluation and laboratory investigations were done, serum survivin level was measured at enrollment in the study as baseline level and after 6 months as follow up level. Results: Serum survivin, ferritin and ESR showed higher levels during activity than that during remission. With even significantly higher levels in MAS group in comparison to SJIA activity groups. Those findings suggest that serum survivin increases with disease chronicity and surges up at time of activity being more significant with secondary MAS development. Ferritin/ESR ratio increases as well with MAS group compared to activity group. The ability of serum survivin, ferritin and ferritin/ESR ratio of secondary MAS prediction in SJIA was studied, they were found to be excellent predictors of secondary MAS, being 100% sensitive and more than 95% specific. Conclusion: The presence of correlations between serum survivin and other markers of activity including ferritin, ESR and ferritin / ESR ratio can support our suggestion about serum survivin as a marker of SJIA activity. These findings can be attributed to the pathogenesis of SJIA as an autoinflammatory disease with continued subtle activation and expansion of macrophage and T-cells. Moreover serum survivin is not only a marker of the cytokine storm that underlies the pathogenesis of SJIA and MAS as well. Serum survivin increases with disease activity and surge up with the development of MAS. Turning serum survivin into an excellent predictor of SJIA secondary MAS, and a very good SJIA activity predictor.