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首页> 外文期刊>International Journal of General Medicine >Overlap of primary biliary cirrhosis and primary sclerosing cholangitis – a rare coincidence or a new syndrome
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Overlap of primary biliary cirrhosis and primary sclerosing cholangitis – a rare coincidence or a new syndrome

机译：原发性胆汁性肝硬化和原发性硬化性胆管炎的重叠-罕见的巧合或新的综合征

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Background: Overlap syndrome has been introduced to the field of hepatology to describe the coexistence of two or more autoimmune hepatic conditions in the same individual. This is a rare clinical case of a patient diagnosed with primary biliary cirrhosis (PBC) who later developed primary sclerosing cholangitis (PSC). This is a unique case as no other cases with a similar pattern have been reported. Overlap syndrome does not include the coexistence of PBC and PSC as a distinctive syndrome so far.Case report: A middle-aged woman suffering from PBC for 17 years got admitted with clinical and biochemical features of cholestatic syndrome. A provisional diagnosis of worsening PBC was proved wrong by magnetic resonance cholangiopancreatography, which revealed typical benign stricture and dilatation of common bile duct with typical beading appearance suggestive of PSC. The patient was stented and treated with an increased dose of ursodeoxycholic acid (UDCA) which improved the symptoms and the biochemical picture.Conclusion: This is a clear overlap of PBC–PSC. It is very difficult to say whether it is a rare coincidence or a new overlap syndrome, but there are no clear guidelines for management of these patients. Currently, the treatment involves endoscopic duct dilatation, UDCA, and regular follow-ups to rule out hepato-biliary tumor occurrence.
机译：背景：重叠综合征已被引入肝病学领域，以描述同一个人中两种或多种自身免疫性肝病的共存。这是诊断为原发性胆汁性肝硬化（PBC）并随后发展为原发性硬化性胆管炎（PSC）的患者的罕见临床病例。这是一个独特的案例，因为没有其他类似案例的报告。迄今为止，重叠综合征尚不包括PBC和PSC并存。病例报告：患有PBC的17岁中年妇女因胆汁淤积综合征的临床和生化特征而入院。磁共振胰胆管造影证实临时诊断为PBC恶化是错误的，该检查显示典型的良性狭窄和胆总管扩张，典型的串珠外观提示PSC。该患者被置入支架并接受增加剂量的熊去氧胆酸（UDCA）治疗，从而改善了症状和生化状况。结论：这与PBC-PSC明显重叠。很难说这是罕见的巧合还是新的重叠综合征，但是对于这些患者的治疗尚无明确的指南。目前，治疗方法包括内窥镜导管扩张术，UDCA和定期随访以排除肝胆肿瘤的发生。

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