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成人斯蒂尔病:28例报告并文献复习

Adult-onset Still's disease: a report of 28 cases and review of the literature.

作者信息

Mehrpoor Golbarg, Owlia Mohammad Bagher, Soleimani Hossein, Ayatollahi Jamshid

机构信息

Department of Internal Medicine, Sadoughi Hospital, Shahid Sadoughi University of Medical Sciences, Safaeieh, Yazd, Iran.

出版信息

Mod Rheumatol. 2008;18(5):480-5. doi: 10.1007/s10165-008-0104-6. Epub 2008 Aug 28.

DOI:10.1007/s10165-008-0104-6
PMID:18754076
Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and outcome of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/microl. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values (>500 ng/ml). The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria.

摘要

成人斯蒂尔病(AOSD)是一种病因不明的罕见全身性炎症性疾病。其特征为发热、皮疹、多关节痛或多关节炎、咽痛、肝脾肿大、淋巴结病、白细胞增多、肝酶升高以及血清铁蛋白水平升高。已有多种皮肤病变在该疾病中被报道。本研究的目的是评估伊朗中部28例AOSD患者的临床和实验室情况。根据AOSD的诊断标准,我们在2002年至2007年间确定了28例患者。我们旨在描述AOSD患者的临床特征、治疗及预后情况。28例AOSD患者中,21例(75%)为女性,7例(25%)为男性。发热(100%)、咽痛(92%)、关节痛(92%)、皮肤划痕症(92%)、典型皮疹(85%)和关节炎(60%)是最常见的表现。实验室检查结果的平均值如下:C反应蛋白(CRP)水平为14.4mg/dl,红细胞沉降率(ESR)为91.5mm/h,白细胞计数为15744.4/微升。25例(89%)患者的天冬氨酸氨基转移酶和丙氨酸氨基转移酶水平异常。20例(71%)患者铁蛋白值升高(>500ng/ml)。临床特征与既往系列报道相似。发热性多关节炎是最常见的表现形式。皮肤划痕症在我们的AOSD患者中是常见现象。鉴于皮肤划痕症是一种简单的诱发性皮肤反应,且在活动性疾病中具有敏感性,我们建议进行更多对照研究以验证其在便捷临床环境中对AOSD诊断的准确性和阳性预测价值,并考虑将其纳入诊断标准。

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