Macrophage activation syndrome in systemic juvenile idiopathic arthritis.

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of systemic juvenile idiopathic arthritis (s-JIA). An immunological feature is the excessive activation and proliferation of T lymphocytes and macrophages. Massive hypercytokinemia is strongly associated with its pathogenesis, particularly the overproduction of interleukin (IL)-1, IL-6 and IL-18; interferon (IFN)-γ; and tumor necrosis factor (TNF)-α. Furthermore, heterozygous mutations in causative genes for primary hemophagocytic lymphohistiocytosis and