Micev M
Sluzba za patohistologiju Institut za bolesti digestivnog sistema KCS, Beograd.
Acta Chir Iugosl. 2000;47(1-2):31-42.
Distinguishing of indeterminate colitis from ulcerative colitis or Crohn's disease in more than 10% of IBD cases as separate diagnostic category (IIBD) is defined by: first, overlapping or paucity of features of two major IBD forms in acute fulminant colitides and few chronic slowly progressive cases without differentiating markers of chronicity, and second, inability to reach specific diagnosis during or after completed clinical, radiological, endoscopical and histopathological examination of chronic colitis with inconclusive presentation of IBD-compatible cases. It implicates the contemporary category of IIBD, until some new relevant data of further clinical (preferably natural history and course of the disease) and histopathological examination of prior biopsies will possibly reclassify 20-75% of these cases into ulcerative colitis category (up to 40%) or Crohn's disease (up to 25%) at a median of 10 years' follow-up. The presence of ileal and/or perianal disease as well as later evident granulomatous and/or fissuring disease, including cases with pouchitis and diversion colitis in addition, could be helpful in favouring of Crohn's disease. Lacking specific signs of disease, IIBD is not a distinct entity. Still, many patients remain in this category. The most far is acute fulminant disease in adolescents and young adults with unusual distribution of transmural inflammation with deep fissuring ulcerations, sometimes of "collar-stud" type and normal mucosa between them, often with relative rectal sparing. There is significant risk of relapse and complications, but mild clinical course and even spontaneous regression are also reported. Failure of ileal pouch-anal anastomosis surgery is about 20% more frequent then in ulcerative colitis with overall worse prognosis in life expectations. Diagnostic problems and the main presentations are discussed in detail, as well as genetic and etiopathogenetic basis for heterogeneity of IBD.
在超过10%的炎症性肠病(IBD)病例中,将不确定性结肠炎与溃疡性结肠炎或克罗恩病区分开来作为一个单独的诊断类别(IIBD)的定义如下:首先,在急性暴发性结肠炎以及少数无慢性特征性标志物的慢性缓慢进展病例中,两种主要IBD形式的特征重叠或缺乏;其次,在对慢性结肠炎进行完整的临床、放射学、内镜和组织病理学检查后,对于表现不明确的IBD兼容病例无法得出明确诊断。这意味着当前的IIBD类别,直到进一步临床(最好是疾病的自然史和病程)以及对先前活检组织进行组织病理学检查的一些新的相关数据可能会在中位10年随访时将这些病例的20%-75%重新分类为溃疡性结肠炎类别(高达40%)或克罗恩病(高达25%)。回肠和/或肛周疾病的存在以及后来明显的肉芽肿性和/或裂隙性疾病,包括伴有袋炎和转流性结肠炎的病例,可能有助于支持克罗恩病的诊断。由于缺乏疾病的特异性体征,IIBD并非一个独特的实体。然而,仍有许多患者属于这一类别。最典型的是青少年和年轻成年人中的急性暴发性疾病,其透壁性炎症分布异常,伴有深部裂隙性溃疡,有时呈“领扣”型,其间黏膜正常,且通常直肠相对 spared。复发和并发症的风险很高,但也有轻度临床病程甚至自发缓解的报道。回肠袋肛管吻合术失败的发生率比溃疡性结肠炎高约20%,总体预期寿命预后更差。详细讨论了诊断问题和主要表现,以及IBD异质性的遗传和病因学基础。