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土耳其色雷斯地区西北部成人斯蒂尔病的流行病学及转归

Epidemiology and outcome of adult-onset Still's disease in Northwestern Thrace region in Turkey.

作者信息

Balci M A, Pamuk Ö N, Pamuk G E, Uzundere F K, Donmez S

机构信息

Department of Rheumatology, Trakya University Medical Faculty, Edirne, Turkey.

Department of Haematology, Trakya University Medical Faculty, Edirne, Turkey.

出版信息

Clin Exp Rheumatol. 2015 Nov-Dec;33(6):818-23. Epub 2015 Aug 31.

PMID:26320744
Abstract

OBJECTIVES

Adult-onset Still's disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. It is characterised by fever, arthritis and, a typical salmon-coloured rash, and is accompanied by fever at nights. Currently, there is limited data on the prevalence of AOSD.

METHODS

Patients diagnosed with AOSD at the Department of Rheumatology of Trakya University Medical Faculty, between 2003 to 2014 were reviewed retrospectively. Patients' clinical features, laboratory measurements, demographics, treatments, follow-up durations, disease courses, outcomes and complications were evaluated.

RESULTS

Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. The most common findings were fever (97.6%), arthralgia (95.2%), arthritis (76.2%), rash (73.8%) and sore throat (40.5%).

CONCLUSIONS

In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.

摘要

目的

成人斯蒂尔病(AOSD)是一种罕见疾病,归类于多因素自身炎症性疾病。其特征为发热、关节炎以及典型的鲑鱼色皮疹,并伴有夜间发热。目前,关于AOSD患病率的数据有限。

方法

回顾性分析2003年至2014年在特拉凯大学医学院风湿病科诊断为AOSD的患者。评估患者的临床特征、实验室检查结果、人口统计学资料、治疗情况、随访时间、疾病进程、结局及并发症。

结果

我们的研究纳入了42例AOSD患者,其中32例(76.2%)为女性,10例(23.8%)为男性(女性与男性比例为3.2)。在研究过程中,AOSD的年发病率为0.62/100,000;总体患病率为6.77/100,000。最常见的表现为发热(97.6%)、关节痛(95.2%)、关节炎(76.2%)、皮疹(73.8%)和咽痛(40.5%)。

结论

在我们基于医院的AOSD研究中,该病的流行病学数据非常有限,我们发现AOSD的发病率显著高于其他系列研究。在我们的系列研究中女性更为常见;在随访时间较长的患者中多循环模式更为常见。

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