A Study of Serum Survivin in Patients with Systemic Onset Juvenile Idiopathic Arthritis

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, hypofibrinogenemia, 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