Kouam C, Warling O, Van Kemseke C, Loly J P, Louis E, Delwaide J
Service d'Hépato-Gastroentérologie et d'Oncologie digestive, CHU Liège, Belgique.
Rev Med Liege. 2020 Nov;75(11):724-730.
Ulcerative ulcerative colitis (UC) is a chronic inflammatory bowel disease characterized by the presence of continuous, uniform, gapless lesions of healthy mucous membrane extending integrally from the anus over a variable portion of the colon. It is a multisystemic pathology for which we will describe one of its hepatic manifestations, primary sclerosing cholangitis (PSC). We present here the story of a patient, a carrier of the Breast Cancer type 1 gene mutation (BRCA1), who initially presented with epigastric and peri-umbilical abdominal pain associated with several episodes of bloody diarrhea. The initial assessment made it possible to diagnose BRCA1 and the fortuitous discovery of biliary tract stenosis of an undetermined nature. Further investigations led to the conclusion that there was a strong presumption of UC associated with PSC or even cholangiocarcinoma (CCA). In the course of treatment, cancer of the gallbladder was also detected. This clinical case makes it possible to review, in the light of recent publications, the pathophysiological link existing between UC and PSC. This article also presents an in-depth analysis of this entity with malignant potential that can affect different sites of the digestive tract, and its own management, which is the subject of new recommendations.
溃疡性结肠炎（UC）是一种慢性炎症性肠病，其特征是从肛门开始在结肠的不同部分存在连续、均匀、无间隙的健康黏膜病变。它是一种多系统疾病，我们将描述其肝脏表现之一，原发性硬化性胆管炎（PSC）。我们在此讲述一名患者的故事，该患者是乳腺癌1型基因突变（BRCA1）携带者，最初表现为上腹部和脐周腹痛，并伴有多次血性腹泻。初步评估得以诊断出BRCA1，并偶然发现性质不明的胆道狭窄。进一步检查得出结论，强烈推测存在与PSC甚至胆管癌（CCA）相关的UC。在治疗过程中，还检测出胆囊癌。结合近期发表的文献，该临床病例有助于回顾UC与PSC之间存在的病理生理联系。本文还对这种具有恶性潜能、可影响消化道不同部位的疾病实体及其自身的管理进行了深入分析，而这正是新建议的主题。
