巨噬细胞活化综合征(MAS)是一种继发于风湿免疫疾病的噬血细胞性淋巴组织细胞增多症(HLH),是一种罕见的、危及生命的过度炎症性疾病。巨噬细胞活化综合征的典型临床症状包括持续高热、皮疹以及肝和脾的肿大,实验室的检测结果显示患者的血细胞数量减少,伴有高甘油三酯或低纤维蛋白原、高铁蛋白血症和转氨酶水平上升等现象。在全身性幼年特发性关节炎(sJIA)和成人发病斯蒂尔氏病(AOSD)中,巨噬细胞活化综合征的发病率较高,但目前有越来越多的学者对系统性红斑狼疮患者发生巨噬细胞活化综合征进行了报道。巨噬细胞活化综合征病情复杂,进展凶险,若误诊或延误治疗时机,可能造成无法挽回的不良结果,所以早期识别巨噬细胞活化综合征并进行诊治就显得十分必要。本文通过检索Pubmed数据库,对系统性红斑狼疮合并巨噬细胞活化综合征的诊治进展做一综述。Macrophage activation syndrome (MAS), a hemophagocytic lymphohistiocytosis (HLH) secondary to rheumatic immune disease, is a rare, life-threatening hyperinflammatory disease. Typical clinical symptoms of macrophage activation syndrome include persistent high fever, rash, and enlargement of the liver and spleen, and laboratory tests show a decrease in the number of blood cells in the patient, with high triglycerides or low fibrinogen, ferritinemia, and elevated aminotransferase levels. The incidence of macrophage activation syndrome is high in systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), but more and more scholars have reported the occurrence of macrophage activation syndrome in patients with systemic lupus erythematosus. Macrophage activation syndrome is complex and progresses dangerously, and if it is misdiagnosed or delayed, it may cause irreversible adverse results, so it is necessary to identify and treat macrophage activation syndrome early. This article reviews the progress
