Department of Gastroenterology with Endoscopy Unit, Medical University of Lublin, 8 Jaczewski Street, 20-954 Lublin, Poland.
Medical University of Warsaw, 61 Zwirki i Wigury Street, 02-091 Warsaw, Poland.
Nutrients. 2023 Feb 2;15(3):760. doi: 10.3390/nu15030760.
Primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are rare immune-related cholangiopathies with still poorly explained pathogenesis. Although triggers of chronic inflammation with subsequent fibrosis that affect cholangiocytes leading to obliteration of bile ducts and conversion to liver cirrhosis are unclear, both disorders are regarded to be multifactorial. Different factors can contribute to the development of hepatocellular injury in the course of progressive cholestasis, including (1) body accumulation of bile acids and their toxicity, (2) decreased food intake and nutrient absorption, (3) gut microbiota transformation, and (4) reorganized host metabolism. Growing evidence suggests that intestinal microbiome composition not only can be altered by liver dysfunction, but in turn, it actively impacts hepatic conditions. In this review, we highlight the role of key factors such as the gut-liver axis, intestinal barrier integrity, bile acid synthesis and circulation, and microbiome composition, which seem to be strongly related to PBC and PSC outcome. Emerging treatments and future therapeutic strategies are also presented.
原发性胆汁性胆管炎（PBC）和原发性硬化性胆管炎（PSC）是罕见的免疫相关胆管疾病，其发病机制仍解释不清。尽管导致慢性炎症和随后纤维化的触发因素尚不清楚，这些因素会影响胆管细胞，导致胆管闭塞和肝硬化转化，但这两种疾病都被认为是多因素的。在进行性胆汁淤积的过程中，导致肝细胞损伤的不同因素包括：（1）胆汁酸在体内的蓄积及其毒性，（2）食物摄入和营养吸收减少，（3）肠道微生物群的转化，和（4）宿主代谢的重新组织。越来越多的证据表明，肠道微生物组组成不仅可以被肝功能障碍改变，而且反过来也可以积极影响肝脏状况。在这篇综述中，我们强调了肠道-肝脏轴、肠道屏障完整性、胆汁酸合成和循环以及微生物组组成等关键因素的作用，这些因素似乎与 PBC 和 PSC 的结果密切相关。还介绍了新兴的治疗方法和未来的治疗策略。
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