Colina Matteo, Govoni Marcello, Trotta Francesco
Section of Rheumatology, Department of Clinical and Experimental Medicine, University of Ferrara, Ferrara, Italy.
Rheumatol Int. 2009 Sep;29(11):1355-7. doi: 10.1007/s00296-008-0813-3. Epub 2008 Dec 2.
Adult-onset Still disease (AOSD) is a rare condition disease of unknown etiology, characterized by quotidian or double quotidian spiking fever, with an evanescent pink-salmon rash, arthritis and multi-organ involvement. Diagnosis is usually clinical and made after other diseases in the differential diagnosis are excluded. We herein report the case of a patient with a remarkable familial autoimmune background in whom adult Still disease started off with a diffuse intravascular coagulation, probably triggered by a macrophage activation syndrome, followed by an acute interstitial myocarditis, leading to a fatal complete atrioventricular block. This case highlights that AOSD represents a troubling condition and that it may suddenly get worse with life-threatening events.
成人斯蒂尔病（AOSD）是一种病因不明的罕见疾病，其特征为每日或隔日出现的高热，伴有一过性的粉红色至鲑鱼色皮疹、关节炎和多器官受累。诊断通常基于临床，在排除鉴别诊断中的其他疾病后做出。我们在此报告一例具有显著家族自身免疫背景的患者，该患者的成人斯蒂尔病最初表现为弥散性血管内凝血，可能由巨噬细胞活化综合征引发，随后出现急性间质性心肌炎，导致致命的完全性房室传导阻滞。该病例突出表明，AOSD是一种棘手的疾病，可能会突然恶化为危及生命的情况。
