Colangitis biliar primaria

E. Buganza-Torio, Christian Navarro-Gerrard
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Abstract

Primary biliary cholangitis is a chronic and progressive inflammatory autoimmune cholestatic liver disease characterized by the destruction of small intrahepatic bile ducts. It is considered a rare disease that predominates in women with a prevalence of 19 to 402 per million people. It is clinically characterized by fatigue, right upper quadrant pain, jaundice, pruritus, and biochemically with alkaline phosphatase (ALP) elevation at least > 1.5 times normal upper limit and the presence of mitochondrial antibody (AMA) positive at titers of 1:40 or more, liver biopsy is necessary in case of high suspicion and negative AMA or in case of suspected overlay syndrome (HAI/CBP). Early diagnosis and treatment of this disease can significantly improve the course of the disease by delaying liver cirrhosis and its complications and impacting patients’ quality of life.
原发性胆道性结肠炎
原发性胆管炎是一种慢性进行性炎症性自身免疫性胆汁淤积性肝病,以肝内小胆管破坏为特征。它被认为是一种罕见的疾病,在妇女中占主导地位,发病率为每百万人19至402人。临床表现为疲劳、右上腹疼痛、黄疸、瘙痒,生化特征为碱性磷酸酶(ALP)升高至少> 1.5倍正常上限,线粒体抗体(AMA)阳性,滴度为1:40及以上,高度可疑且AMA阴性或疑似覆盖综合征(HAI/CBP)需行肝活检。早期诊断和治疗可通过延缓肝硬化及其并发症,影响患者的生活质量,显著改善病程。
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