Macrophage activation syndrome in systemic juvenile idiopathic arthritis: analysis of abnormalities in interferon-gamma (IFN-g) and critical role of suppressor of cytokine signaling-m (SOC-3). KU Leuven
Systemic juvenile idiopathic arthritis (sJIA) is a severe childhood immune-inflammatory disorder characterized by arthritis, fever, rash and lymphadenopathy. About 10% of sJIA patients develop a life-threatening complication called macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis (HLH). Both disorders are characterized by a cytokine storm, with an important role for interleukin (IL)-1β, IL-6 and ...