不明原因发热患者中的成人型斯蒂尔病:一个罕见病例

Silky Kumari, Subhankar Das, Sudha Kumari, Gyan Ranjan, Mahaprasad Barik, Ramya Ranjan Kanta, Somnath Nishad
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引用次数: 0

摘要

成人斯蒂尔病(Adult onset Still's disease,AOSD)是一种病因不明的罕见临床病,以关节炎、发热、皮疹和其他全身症状为特征。AOSD 一般不会与其他风湿性疾病重叠。AOSD 是一种排除性诊断,也是不明原因发热(PUO)的常见病因之一,如果治疗不当,可能会危及生命。虽然类固醇是第一线治疗方法,但约有 20%-30% 的患者对类固醇难治、不耐受,或在逐渐减量或停用类固醇后复发。目前还没有治疗这类患者的临床指南,因此具有挑战性。关键词 斯蒂尔病、关节炎、发热、自身炎症性
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Adult Onset Still’s Disease in a Patient with Fever of Unknown Origin: A Rare Case
Adult onset Still’s disease (AOSD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, evanescent rash, and other systemic presentations. AOSD generally does not overlap with other rheumatic diseases. AOSD is a diagnosis by exclusion, and it is one of the common causes of pyrexia of unknown origin (PUO) which can be life-threatening if mistreated. Although steroids are the first line of therapy, about 20%-30% of patients are refractory, intolerant, and or relapse during tapering or upon discontinuation of steroids. There are no clinical guidelines in treating such patients, which is challenging. Keywords:  Still’s disease, arthritis, fever, auto-inflammatory
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