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[白塞病的肠道表现]

[Intestinal manifestations of Behçet's disease].

作者信息

Goloeva R G, Alekberova Z S, Lisitsyna T A, Stepanova E A, Orlova L P

机构信息

V.A. Nasonova Scientific and Research Institute of Rheumatology.

V.M. Buyanova Municipal clinical hospital.

出版信息

Ter Arkh. 2019 May 15;91(5):111-119. doi: 10.26442/00403660.2019.05.000247.

Abstract

Behçet's disease (BD) is a systemic vasculitis of unknown origin, characterized by recurrences of the ulcerative process in the oral cavity and on the genitals, inflammatory damage of the eyes, joints, vessels and other organs. The severity and prognosis of BD determines organ pathology. Intestinal manifestations of BD (intestinal BD) are the least studied. Its verification in BD is complicated by the variety of clinical manifestations, their similarity with inflammatory bowel diseases, the lack of informative laboratory tests, pathognomonic endoscopic and histological signs. Intestinal BD can lead to serious complications (massive bleeding, intestinal perforation and fistula formation), which can not only significantly reduce the quality of the patient's life, but also cause death. Treatment of intestinal BD is not standardized; it is mainly empirical and conducted courses. The purpose of therapy is to achieve clinical remission, healing of intestinal ulcers and prevention of surgery. The article presents a case of severe refractory intestinal BD, requiring twice emergency surgical care - removal of half and then the whole of the colon because of multiple perforations. A brief review of the literature is given and diagnostic difficulties of intestinal BD are discussed.

摘要

白塞病(BD)是一种病因不明的系统性血管炎,其特征为口腔和生殖器反复出现溃疡性病变、眼睛、关节、血管及其他器官发生炎性损伤。白塞病的严重程度和预后决定了器官病变情况。白塞病的肠道表现(肠道白塞病)研究最少。在白塞病中对其进行确诊较为复杂,这是因为临床表现多样、与炎症性肠病相似、缺乏有诊断价值的实验室检查、特征性的内镜及组织学表现。肠道白塞病可导致严重并发症(大量出血、肠穿孔和瘘管形成),这不仅会显著降低患者生活质量,还可能导致死亡。肠道白塞病的治疗并不规范;主要是经验性治疗且分疗程进行。治疗目的是实现临床缓解、肠道溃疡愈合及预防手术。本文介绍了一例严重难治性肠道白塞病病例,因多处穿孔先后两次进行急诊手术,先切除半结肠,后切除整个结肠。文中还对相关文献进行了简要综述,并讨论了肠道白塞病的诊断难点。

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