Systemic Juvenile Idiopathic Arthritis: Diagnosis and Management.

Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. In sJIA, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of sJIA and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. Recent data suggests that early cytokine blockage might abrogate chronic, destructive, therapy resistant arthritis phase, reflecting a potential "window of opportunity" in the care of children with sJIA.