Синдром активации макрофагов у больных системным ювенильным артритом

摘要: Macrophage activation syndrome (MAS) is one of the histiocytic diseases developing from cells a macrophage series, hemophagocytic lymphohis- tiocytosis (HLH). Rheumatic have been demonstrated to be often associated with development SAM, most upon systemic juve- nile arthritis (SJA). Certain issues discussed concerning pathogenesis concept defect mechanisms T-cell cytotoxicity and reduction activity level natural killer (NK) cells, which are mutation in PRF1 gene encoding perforin, as well overproduction, by T-lymphocytes histiocytes, number cytokines (interleukin 1 β – IL1 , interferon γ tumor necrosis factor α soluble IL2-receptor), indirectly lead tissue macrophages production proinflammatory cytokines. The diagnosis problems low sensitivity specificity HLH 2010 diagnostic criteria for syndrome, based on molecular genetics pathomorphological HLH, discussed. (2012) developed SJA presented. Thrombocytopenia, hyperferritinemia, pathohistological signs hemophagocytosis greatest significance. Attention paid need subclinical mild forms identification potential risk groups, prevention SAM development. differential considered allowance similarity clinical manifestations SJA, treat- ment tactics using 2004 protocol, biological therapy.