文献简介

出版社:Macmillan Publishers Limited

作  者:Nicola L. Ambrose and Dorian O. Haskard

编  号:

关键字:Behçet syndrome,diagnosis and management

年  份:2013   点击量:878

文献摘要 全文翻译

Abstract 
Behçet syndrome (also known as Behçet disease) is a rare condition that is associated with considerable morbidity. Cases of Behçet syndrome have been reported worldwide, but the highest prevalence occurs in countries that border the ancient Silk Route, such as Turkey and Iran. Although oral ulceration, genital ulceration and eye disease are the classic triad of manifestations, the cardiovascular, gastrointestinal, musculoskeletal and central nervous systems can also be affected. The syndrome is chronic and relapsing with some patients having benign episodes whereas others have more serious complications, including blindness or the rupture of a pulmonary arterial aneurysm. Diagnosing Behçet syndrome, particularly outside of endemic regions, often incurs a considerable delay owing to the rarity of this condition. Furthermore, a paucity exists of data from randomized controlled trials on the optimal therapeutic approaches to use in patients, as well as a lack of informative laboratory surrogate markers to monitor disease progression. This Review discusses the issues surrounding the diagnosis and differential diagnosis of Behçet syndrome and presents the current approaches to managing patients with this complex group of disorders.  

摘要
Behçet综合征(又称白塞病)是一种罕见的疾病,具有相当高的发病率。世界各地均有报道Behçet综合征的病例,但其发病率最高的是在古丝绸之路边境上的国家,如土耳其和伊朗。尽管口腔溃疡、生殖器溃疡和眼部疾病是其三种典型的临床表现,但是心血管、胃肠道、肌肉骨骼以及中枢神经系统也可受累。Behçet综合症是一种慢性、复发性的疾病,一些患者为良性病程,而另一些患者则有严重的并发症,如失明或肺动脉动脉瘤破裂。对于Behçet综合征的诊断,尤其是在非流行地区,常常因为该疾病非常罕见,而耽误对其诊断。此外,缺乏采用最佳治疗方法的随机对照试验的患者数据,以及有效的实验室替代指标来监测疾病的进展。本文围绕着对Behçet综合征的诊断和鉴别诊断进行了讨论,并提出了当前对这种复杂性疾病的治疗方法。