Intestinal Behqet's disease presenting as a massive acute lower gastrointestinal bleed
Intestinal Behqet's disease presenting as a massive acute lower gastrointestinal bleed
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DOI：
10.1023/a:1017999515606
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发表时间：
2002-03-01
影响因子：
3.1
通讯作者：
Siddiqui, D
中科院分区：
文献类型：
--
作者：
Smith, JA;Siddiqui, D
白塞氏病最初于 1937 年被描述为一种涉及口腔和生殖器溃疡以及眼部炎症的综合征 (1)。从那时起，白塞氏病被认为是一种系统性血管炎，可以同时或在不同时间影响身体的多个器官。虽然它可能有急性表现，但其粗糙通常是惰性和慢性的。其病因尚不清楚，但主要的病因理论包括感染、自身免疫和遗传机制 (2)。不同地理位置的患病率差异很大。白塞氏病最常见于从远东延伸至地中海盆地的古代丝绸之路沿线。在日本，患病率为十万分之十，沙特阿拉伯为十万分之二十，而在北欧和美国，患病率仅为十万分之 0.3。男女比例也因地理位置而异。在埃及、土耳其、以色列和伊朗，男性占主导地位，而在欧洲、美国和日本，女性占主导地位。发病年龄范围从婴儿期到 70 多岁，但最高频率发生在 30 岁和 40 岁之间 (3)。 Bechguard 于 1940 年首次描述了胃肠道受累 (4)。 Oshima 等人报道，超过 40% 的白塞氏病患者有胃肠道不适。症状包括腹痛、腹泻、恶心、厌食和腹胀 (5)。尽管胃肠道症状很常见，但胃肠道溃疡的表现却很少见。这种所谓的肠道白塞氏病仅占病例的大约 1% (6, 7)。
Behçet's disease was originally described in 1937 as a syndrome involving oral and genital ulceration and ocular inflammation (1). Since this time Behçet's disease has been recognized as a systemic vasculitis that can affect multiple organs of the body concurrently or at variable times. Although it may have an acute presentation, its coarse is usually indolent and chronic. Its cause is unknown, but the leading etiologic theories include infectious, autoimmune, and genetic mechanisms (2).The prevalence varies widely between geographic locations. Behçet's disease occurs most commonly along the ancient silk road which extends from the Far East to the Mediterranean basin. In Japan the prevalence is 10 in 100,000, in Saudi Arabia it is 20 in 100,000, and in Northern Europe and in the United States it is only 0.3 per 100,000. The male-to-female ratio also varies by geographic location. Men predominate in Egypt, Turkey, Israel, and Iran, whereas women predominate in Europe, the United States, and Japan. The age of onset can range from infancy to the 70s, although the highest frequency occurs in the third and fourth decades (3). Bechguard first described gastrointestinal involvement in 1940 (4). Oshima et al reported that over 40% of Behçet's disease patients had gastrointestinal complaints. Symptoms included abdominal pain, di-arrhea, nausea, anorexia, and abdominal distension (5). Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal Behçet's disease ac-counts only for approximately 1% of cases (6, 7).