Systemic Juvenile Idiopathic Arthritis: A Review

CME Educational Objectives 1. Become familiar with the clinical presentation, differential diagnosis and potential complications of systemic juvenile idiopathic arthritis. 2. Understand what is known about the pathogenesis of systemic juvenile idiopathic arthritis and role of inflammatory cytokines in this disease. 3. Know the available treatments and current algorithm for treatment escalation in systemic juvenile idiopathic arthritis. Systemic juvenile idiopathic arthritis is a subtype of juvenile idiopathic arthritis, according to the recent International League of Associations for Rheumatology diagnostic criteria. 1 In North America and Europe, systemic juvenile idiopathic arthritis accounts for 5% to 15% of children diagnosed with juvenile idiopathic arthritis, although the disease is seen worldwide. 2 This review will summarize the clinical manifestations, pathogenesis, treatment, and prognosis of systemic juvenile idiopathic arthritis. It will also review one of the most interesting and feared complications of the disease, macrophage activation syndrome.