Department of Internal Medicine, Hôpital de la Croix-Rousse, Université Claude Bernard-Lyon 1, Lyon, France ; Department of Biochemistry, University of Lausanne, Epalinges, Switzerland ; International Research Center on Infectiology. INSERM U1111. Université Claude Bernard-Lyon 1, Lyon, France.
Department of Internal Medicine, Hôpital de la Croix-Rousse, Université Claude Bernard-Lyon 1, Lyon, France.
Ther Clin Risk Manag. 2014 Dec 22;11:33-43. doi: 10.2147/TCRM.S64951. eCollection 2015.
Adult-onset Still's disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. The former classification, based on the disease course, seems to be quite dated. Indeed, there is accumulating evidence that AOSD can be divided into two distinct phenotypes based on cytokine profile, clinical presentation, and outcome, ie, a "systemic" pattern and an "articular" pattern. The first part of this review deals with the treatments that are currently available for AOSD. We then present the different strategies based on the characteristics of the disease according to clinical presentation. To do so, we focus on the two subsets of the disease. Finally, we discuss the management of life-threatening complications of AOSD, along with the therapeutic options during pregnancy.
成人Still 病（AOSD）是一种罕见的炎症性疾病，最近被归类为一种多基因自身炎症性疾病。以前的分类，基于疾病过程，似乎已经相当过时。事实上，越来越多的证据表明，AOSD 可以根据细胞因子谱、临床表现和结果分为两种不同的表型，即“全身性”和“关节性”。这篇综述的第一部分涉及目前可用于 AOSD 的治疗方法。然后，我们根据临床表现提出了基于疾病特征的不同策略。为此，我们重点介绍了该疾病的两个亚组。最后，我们讨论了 AOSD 危及生命的并发症的处理以及妊娠期间的治疗选择。
