Division of Rheumatology, University of L'Aquila, L'Aquila, Italy.
Division of Rheumatology, University of Palermo, Palermo, Italy.
Autoimmun Rev. 2017 Jan;16(1):16-21. doi: 10.1016/j.autrev.2016.09.016. Epub 2016 Sep 21.
Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokine storm, which is involved in the development of multiple organ failure. In this paper, we investigated clinical data, treatments, and outcome of a homogeneous cohort of 41 adult MAS patients, complicating autoimmune rheumatic diseases. MAS-related death occurred in 17 patients (42.5%) during the follow-up, and older age and increased serum ferritin levels, at the time of diagnosis, were significantly associated with mortality. In conclusion, adult MAS is associated with high mortality rate. Some clinical features at diagnosis may be predictive of MAS-associated death.
巨噬细胞活化综合征（MAS）是一种罕见的、危及生命的疾病，早期诊断和积极的治疗策略可能改善预后。由于其罕见性，流行病学数据仍然缺乏。高血清铁蛋白血症常与 MAS 相关，并可能调节细胞因子风暴，这与多器官衰竭的发生有关。在本文中，我们研究了 41 例合并自身免疫性风湿病的成人 MAS 患者的临床资料、治疗和预后。在随访期间，17 例患者（42.5%）发生 MAS 相关死亡，诊断时的年龄较大和血清铁蛋白水平升高与死亡率显著相关。总之，成人 MAS 死亡率高。一些诊断时的临床特征可能是 MAS 相关死亡的预测因素。
