Macrophage activation syndrome: A challenge for pediatrician from rheumatology department and pediatric intensive care unit / 中国小儿急救医学

ABSTRACT

Macrophage activation syndrome(MAS)is a potentially life-threatening complication of rheumatic disorders.Macrophage activation-like syndrome refers to sepsis patient with dominant pro-inflammatory mechanism of organ dysfunction.Decreased cytolytic function results in prolonged interaction of the effective lymphocytes and their target antigen presenting cells, thus resulting in the pro-inflammatory cytokine storm which lead to multi-organ systerm failure.Early diagnosis remains challenging even there is a set of universal diagnostic criteria.Intravenous pulse methylprednisolon combination with cyclosporin A is still the mainstream therapeutic protocol in domestic.Novel cytokine-targeted approaches are being explored for safer and effective therapies for children with MAS.