Division of Gastroenterology and Hepatology, University Hospital Zurich, 8091 Zurich, Switzerland.
J Crohns Colitis. 2011 Feb;5(1):64-8. doi: 10.1016/j.crohns.2010.09.003. Epub 2010 Oct 29.
Cogan's syndrome (CS) is a rare autoimmune disease with less than 250 cases reported. It mainly affects the audiovestibular system and the eyes frequently resulting in deafness. Inflammatory bowel disease (IBD) consists of two subtypes, Crohn's disease (CD) and ulcerative colitis (UC), and represents a common form of chronic intestinal inflammation. Here, we report an association between CS and IBD in four patients.
Patient data were collected using a questionnaire that was sent to one of our outpatients and three of the 13 members of the German CS self-help group.
In all Cogan patients with IBD (3 female with UC, 1 male with CD), intestinal disease was diagnosed years before the onset of CS. After suffering from a complicated IBD disease course, they suddenly developed CS-related symptoms, such as hearing loss, tinnitus or eye inflammation. Three of them went deaf within a few years after diagnosis. Although all of them had been on immunosuppressive IBD therapy, these treatment regimens did not prevent the onset of CS.
Our data suggest a strong association of IBD and CS. Since CS rapidly leads to bilateral deafness, it seems to be a rare, but nevertheless important disease that can occur in association with IBD. However, neither an early diagnosis nor an immunosuppressive therapy seems to efficaciously prevent disease progression.
Cogan 综合征（CS）是一种罕见的自身免疫性疾病，报告的病例少于 250 例。它主要影响听觉和视觉系统，常导致耳聋。炎症性肠病（IBD）包括两种亚型，克罗恩病（CD）和溃疡性结肠炎（UC），是一种常见的慢性肠道炎症形式。在这里，我们报告了 4 例 CS 与 IBD 之间的关联。
通过向我们的一名门诊患者和德国 CS 自助小组的 13 名成员中的 3 名成员发送问卷来收集患者数据。
所有患有 IBD 的 Cogan 患者（3 名女性患有 UC，1 名男性患有 CD），在 CS 发病前多年就被诊断出患有肠道疾病。在经历了复杂的 IBD 病程后，他们突然出现了与 CS 相关的症状，如听力损失、耳鸣或眼部炎症。其中 3 人在诊断后几年内失聪。尽管他们都接受了免疫抑制性 IBD 治疗，但这些治疗方案并未阻止 CS 的发生。
我们的数据表明 IBD 和 CS 之间存在很强的关联。由于 CS 迅速导致双侧耳聋，因此它似乎是一种罕见但很重要的疾病，可与 IBD 相关发生。然而，无论是早期诊断还是免疫抑制治疗似乎都不能有效地阻止疾病进展。
