Macrophage activation syndrome in a case of systemic onset juvenile idiopathic arthritis: Diagnostic dilemmas

Macrophage activation syndrome or hemophagocytic lymphohistiocytosis is a heterogeneous group of disorders characterized by activated macrophage activity leading to a sepsis-like clinical presentation with hemophagocytosis, hyperferritinemia, hypercytokinemia, and variable cytopenias often resulting in multiple organ failure, occurs much more frequently in individuals with systemic onset juvenile idiopathic arthritis.