Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea.
Yonsei Med J. 2013 Sep;54(5):1084-90. doi: 10.3349/ymj.2013.54.5.1084.
Behçet's disease (BD) is a multisystem inflammatory disorder that presents as recurrent oral and genital ulcers in conjunction with other dermatological and ocular manifestations. The prevalence of BD is higher in Middle and East Asia than in Western countries. Intestinal BD is a specific subtype of BD, characterized by intestinal ulcers and associated gastrointestinal symptoms. Similar to inflammatory bowel disease, intestinal BD exhibits a fluctuating disease course with repeated episodes of relapse and remission that necessitate adequate maintenance therapy after achievement of clinical remission. Medical treatment of intestinal BD is largely empirical since well-controlled studies have been difficult to perform due to the heterogeneity and rarity of the disease. To date, 5-aminosalicylic acid, systemic corticosteroids, and immunosuppressants have been used anecdotally to treat intestinal BD. The clinical course of intestinal BD shows considerable variability, and the exact point at which more potent agents such as immunosuppressants should be used has not yet been elucidated. Given the difficulty in predicting which patients will experience complicated disease courses and the fact that these drugs are related with certain risk resulting from immunosuppression, proper identification of prognostic factors in intestinal BD may allow physicians to implement tailored medical therapy and individualized patient monitoring based on risk stratification. In this review, the impact of baseline characteristics on the long-term course of intestinal BD, prognostic factors during various medical therapies, and outcome predictors related to surgery will be discussed.
贝赫切特病(BD)是一种多系统炎症性疾病,表现为复发性口腔和生殖器溃疡,并伴有其他皮肤和眼部表现。BD 的患病率在中、东亚高于西方国家。肠 BD 是 BD 的一种特殊亚型,其特征为肠溃疡和相关的胃肠道症状。与炎症性肠病相似,肠 BD 表现为疾病反复发作和缓解的波动过程,在达到临床缓解后需要进行充分的维持治疗。由于疾病的异质性和罕见性,肠 BD 的医学治疗在很大程度上是经验性的,因为难以进行良好对照的研究。迄今为止,5-氨基水杨酸、全身皮质类固醇和免疫抑制剂已被用于治疗肠 BD。肠 BD 的临床病程表现出很大的可变性,尚未阐明何时应使用更有效的药物(如免疫抑制剂)。鉴于预测哪些患者会经历复杂的疾病过程存在困难,并且这些药物与免疫抑制相关的某些风险有关,因此,在肠 BD 中正确识别预后因素可能使医生能够根据风险分层实施个体化的医学治疗和个体化的患者监测。在这篇综述中,将讨论基线特征对肠 BD 长期病程的影响、各种医学治疗期间的预后因素以及与手术相关的结果预测因素。