Università degli Studi di Genova, Genoa, Italy.
Università degli Studi di Napoli Federico II, Napoli, Italy.
Pediatr Allergy Immunol. 2020 Feb;31 Suppl 24:13-15. doi: 10.1111/pai.13158.
Macrophage activation syndrome (MAS) is a serious, potentially life-threatening, hyperinflammatory condition, which belongs to the spectrum of hemophagocytic lymphohistiocytosis (HLH) and can complicate several immunologic and rheumatic disorders. MAS is characterized by a dysfunctional immune response that is similar to that seen in other forms of HLH. Because MAS may pursue a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are fundamental. Recently, a set of classification criteria for MAS complicating sJIA has been developed through a multinational collaborative effort. High-dose parenteral corticosteroids remain the mainstay of treatment of MAS.
巨噬细胞活化综合征 (MAS) 是一种严重的、潜在危及生命的、炎症过度活跃的疾病，属于噬血细胞性淋巴组织细胞增生症 (HLH) 谱的一种，并可使几种免疫和风湿性疾病复杂化。MAS 的特征是免疫反应功能障碍，类似于其他形式的 HLH。由于 MAS 可能会迅速导致致命的后果，因此迅速识别其临床和实验室特征并立即进行治疗干预是至关重要的。最近，通过多国合作努力，制定了一套用于 sJIA 合并 MAS 的分类标准。大剂量静脉内皮质类固醇仍然是 MAS 治疗的主要方法。
