AB2 Bio - Secondary HLH Protein

Macrophage Activation Syndrome. Macrophage Activation Sysdrome (MAS) is a very severe complication of rheumatic diseases. Mortality ranges between 10-30% for patients with MAS and there is no registered therapy. MAS occurs frequently in systemic Juvenile Idiopathic Arthritis (soJIA) and in Adult onset Still’s Disease (AOSD) patients. It is characterized by severe systemic inflammation with pancytopenia, liver insufficiency, coagulopathy and neurological symptoms. The underlying cellular mechanism is the uncontrolled activation and proliferation of T lymphocytes and macrophages leading to a cytokine storm with a predominant role of IL-18.