巨噬细胞活化综合征的再认识

Recognition of macrophage activation syndrome

巨噬细胞活化综合征（macrophage activation syndrome，MAS）是一种严重的具有潜在生命威胁的、多继发于幼年特发性关节炎全身型（systemic onset juvenile idiopathic arthritis，sJIA）失控的自身炎症性反应综合征。其临床表现包括持续发热、肝、脾淋巴结大、血细胞减少、高铁蛋白血症、低纤维蛋白原血症、高三酰甘油血症、凝血功能障碍以及骨髓中可见吞噬血细胞。现总结继发于sJIA的MAS的临床特征，并对其诊断和治疗上的新进展进行综述。

Macrophage activation syndrome （MAS） is a serious potentially life -threatening complication seen primarily in patients with systemic onset juvenile idiopathic arthritis （sJIA）. Clinical symptoms include persistent fever, liver and spleen lymph node enlargement, cytopenia, hyperferritinemia, hypofibrinogenemia, hypertriglyceri- demia, coagulation disorders, and hemophagocytosis can be seen in the bone marrow. This article summarizes the characteristics of MAS occurring in the context of sJIA and discuss the recent advances in classification systems and nlanazement.