a Division of Rheumatology , University of L'Aquila , L'Aquila , Italy.
b Division of Rheumatology , University of Palermo , Palermo , Italy.
Expert Rev Clin Immunol. 2017 Nov;13(11):1041-1047. doi: 10.1080/1744666X.2017.1372194. Epub 2017 Sep 1.
Macrophage activation syndrome (MAS) is a severe, hyperinflammatory life-threatening syndrome, generally complicating different rheumatic diseases. Despite the severity of the disease, little is known about the pathogenic mechanisms and, thus, possible targeted therapies in the management of these patients. Areas covered: In this review, we aimed to update the current pathogenic knowledge of MAS, during rheumatic diseases, focusing mainly on immunologic abnormalities and on new possible therapeutic strategies. Expert commentary: The difficult pathogenic scenario of MAS, in which genetic defects, predisposing diseases, and triggers are mixed together with the high mortality rate, make it difficult to manage these patients. Although most efforts have been focused on investigating the disease in children, in recent years, several studies are trying to elucidate the possible pathogenic mechanism in adult MAS patients. In this context, genetic and immunological studies might lead to advances in the knowledge of pathogenic mechanisms and possible new therapeutic targets. In the future, the results of ongoing clinical trials are awaited in order to improve the management and, thus, the survival of these patients.
巨噬细胞活化综合征（MAS）是一种严重的、炎症过度活跃的危及生命的综合征，通常并发于不同的风湿性疾病。尽管这种疾病很严重，但人们对其发病机制知之甚少，因此在这些患者的治疗中可能缺乏靶向治疗。
在这篇综述中，我们旨在更新 MAS 在风湿性疾病中的当前发病机制知识，主要关注免疫异常和新的可能治疗策略。
MAS 的发病机制复杂，遗传缺陷、易患疾病和诱因交织在一起，且死亡率很高，这使得这些患者的治疗变得困难。尽管大多数努力都集中在研究儿童中的疾病，但近年来，一些研究试图阐明成人 MAS 患者的可能发病机制。在这方面，遗传和免疫研究可能会促进对发病机制的认识，并为可能的新治疗靶点提供依据。未来，人们期待正在进行的临床试验的结果，以改善这些患者的治疗，从而提高他们的生存率。
