Primary biliary cholangitis in patients with inflammatory bowel disease
Primary biliary cholangitis in patients with inflammatory bowel disease
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DOI：
10.1016/j.clinre.2019.05.002
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发表时间：
2020-02-01
影响因子：
2.7
通讯作者：
Macedo, Guilherme
中科院分区：
文献类型：
--
作者：
Liberal, Rodrigo;Gaspar, Rui;Macedo, Guilherme
背景与目的：炎症性肠病（IBD）与各种肝胆系统疾病有关。与原发性硬化性胆管炎（PSC）密切相关。原发性胆汁性胆管炎（PBC）是另一种自身免疫性胆汁淤积性肝脏疾病，通常与IBD无关。本研究的目的是报告PBC相关IBD的病例，其临床特征，对治疗的反应和长期outcome.Methods：回顾性分析了2006年至2016年识别出的PBC相关IBD患者的前瞻性整理数据库。PBC已根据公认的标准进行诊断，并根据Ludwig分类进行分期。进行磁共振胰胆管造影（MRCP）以排除PSC。熊去氧胆酸（UDCA）治疗的反应进行了评估，通过使用巴黎II criteria.Results：共6例患者（5名女性）与PBC相关的IBD被确定。IBD诊断时的中位年龄为44.7岁（范围22.5-48.8岁）。三人患有克罗恩病，三人患有溃疡性结肠炎。PBC均在IBD确诊后确诊。所有患者均表现为胆汁淤积，抗线粒体抗体均阳性。MRCP均正常。肝活检与PBC一致（1例I期，5例II期）。一年后开始UDCA，所有患者对therapy.Conclusion：在此，我们描述了迄今为止报道的最大系列的PBC相关IBD。虽然这两种情况的共存是罕见的，PBC应考虑在IBD患者肝功能检查异常的工作。(C)2019年由Elsevier Masson SAS出版。
Background & Aims: Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders. There is a strong association for primary sclerosing cholangitis (PSC). Primary biliary cholangitis (PBC), another autoimmune cholestatic liver disease, is not usually associated with IBD. The aim of this study is to report cases of PBC-associated IBD, their clinical features, response to therapy, and long-term outcome.Methods: Retrospective analysis of a prospectively collated database identified patients presenting with PBC-associated IBD from 2006 to 2016. PBC has been diagnosed according to accepted criteria, and staged according to Ludwig classification. A magnetic resonance cholangiopancreatography (MRCP) was performed to rule out PSC. Response to ursodeoxycholic acid (UDCA) therapy has been assessed by using the Paris II criteria.Results: A total of six patients (five females) with PBC-associated IBD were identified. Median age at IBD diagnosis was 44.7 years (range 22.5-48.8). Three had Crohn's disease and three ulcerative colitis. PBC was diagnosed in all after IBD had been diagnosed. All patients presented with cholestasis, all were positive for anti-mitochondrial antibodies. MRCP was normal in all. Liver biopsy was consistent with PBC (stage I in one, stage II in five). One year after initiation of UDCA, all patients responded to therapy.Conclusion: Herein we describe the largest series reported to date of PBC-associated IBD. Although the coexistence of the two conditions is rare, PBC should be considered during the work-up of abnormal liver function tests in patients with IBD. (C) 2019 Published by Elsevier Masson SAS.