Elbirt Daniel, Asher Ilan, Sthoeger Zev M
Dep. of Internal Medicine B, Kaplan Medical Center, Rehovot.
Harefuah. 2002 May;141(5):462-7, 497.
Behçet's disease is a complex, multisystem disease first described in 1937 by the Turkish dermatologist, Hulusi Behçet. In his original description, Behçet referred to a symptom complex of recurrent oral aphthous ulcers, genital aphthae, and iritis that could lead to blindness. Today we know that Behçet's disease is much more than a "triad syndrome" and it may also involve the gastrointestinal, central nervous system and large vessels. As yet, there are no existing diagnostic laboratory tests or curative treatments for Behçet's disease. Howver, genetic studies have identified those who are at risk and newer molecular biologic investigations further elucidate the pathogenesis of the disease. The treatment of Behçet's disease depends on clinical manifestations and the severity of the disease. Although various therapeutic modalities have been employed for Behçet's disease, treatment is still unsatisfactory. Treatment modalities include local, systemic, or surgical approaches. Only limited success has been achieved with classic agents such as: colchicine, indomethacin, chlorambucil, and systemic corticosteroids. New therapeutic approaches have been introduced for Behçet's disease using cyclosporine, thalidomide, interferon, high-dose corticosteroids or cyclophosphamide therapy. In this article we summarized the clinical and diagnostic approach for Behçet's disease and the current therapy indicated for different clinical presentations of the disease.
白塞病是一种复杂的多系统疾病,1937年由土耳其皮肤科医生胡卢西·白塞首次描述。在他最初的描述中,白塞提到了一种复发性口腔溃疡、生殖器溃疡和虹膜炎的症状复合体,这些症状可能导致失明。如今我们知道,白塞病远不止是一种“三联征综合征”,它还可能累及胃肠道、中枢神经系统和大血管。目前,尚无针对白塞病的诊断性实验室检查或治愈性治疗方法。然而,基因研究已经确定了那些有患病风险的人,新的分子生物学研究进一步阐明了该疾病的发病机制。白塞病的治疗取决于临床表现和疾病的严重程度。尽管已经对白塞病采用了各种治疗方式,但治疗效果仍然不尽人意。治疗方式包括局部、全身或手术方法。使用秋水仙碱、吲哚美辛、苯丁酸氮芥和全身用皮质类固醇等传统药物仅取得了有限的成功。已经引入了使用环孢素、沙利度胺、干扰素、大剂量皮质类固醇或环磷酰胺治疗白塞病的新治疗方法。在本文中,我们总结了白塞病的临床和诊断方法以及针对该疾病不同临床表现的当前治疗方法。