Purpose of review: Macrophage activation syndrome is a life-threatening complication seen predominantly in children with systemic onset juvenile idiopathic arthritis. It accounts for a significant amount of the morbidity and mortality seen with systemic onset juvenile idiopathic arthritis.
Recent findings: In this article, we will look at the new developments in the diagnosis, classification, pathogenesis and management of macrophage activation syndrome in systemic onset juvenile idiopathic arthritis patients.
Summary: More work is needed to further elucidate the pathophysiology of macrophage activation syndrome in systemic onset juvenile idiopathic arthritis. This would be the key to early diagnosis using more sensitive criteria and better management.