Rheumatology Unit, S. Anna Hospital and University of Ferrara, Italy.
Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy.
Autoimmun Rev. 2017 Oct;16(10):1016-1023. doi: 10.1016/j.autrev.2017.07.017. Epub 2017 Aug 1.
Adult onset Still's disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by four cardinal signs which are almost always present in patients: high spiking fever, arthralgia (with or without synovitis), maculo-papular salmon-pink evanescent skin rash, striking leukocytosis with neutrophilia. Here, we review the clinical features of AOSD and describe the best practice approaches for its management, reviewing available guidelines and recommendations and providing experts' insights.
成人斯蒂尔病（AOSD）是一种罕见的病因不明的全身炎症性疾病，其特征为几乎所有患者均存在的四项主要表现：高热、关节炎（伴或不伴滑膜炎）、一过性鲑鱼红斑斑丘疹样皮疹、显著的白细胞增多伴中性粒细胞增多。在此，我们回顾了 AOSD 的临床特征，并描述了其治疗的最佳实践方法，同时回顾了现有指南和建议，并提供了专家的见解。
