Department of Medicine III, University Hospital RWTH Aachen, Aachen, 52074, Germany.
Norwegian PSC Research Center, Section of Gastroenterology and Research Institute of Internal Medicine, Division of Surgery, Inflammatory Diseases and Transplantation, Oslo University Hospital and University of Oslo, Oslo, Norway.
Nat Commun. 2023 Jun 6;14(1):3304. doi: 10.1038/s41467-023-38840-8.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by chronic inflammation and progressive fibrosis of the biliary tree. The majority of PSC patients suffer from concomitant inflammatory bowel disease (IBD), which has been suggested to promote disease development and progression. However, the molecular mechanisms by which intestinal inflammation may aggravate cholestatic liver disease remain incompletely understood. Here, we employ an IBD-PSC mouse model to investigate the impact of colitis on bile acid metabolism and cholestatic liver injury. Unexpectedly, intestinal inflammation and barrier impairment improve acute cholestatic liver injury and result in reduced liver fibrosis in a chronic colitis model. This phenotype is independent of colitis-induced alterations of microbial bile acid metabolism but mediated via hepatocellular NF-κB activation by lipopolysaccharide (LPS), which suppresses bile acid metabolism in-vitro and in-vivo. This study identifies a colitis-triggered protective circuit suppressing cholestatic liver disease and encourages multi-organ treatment strategies for PSC.
原发性硬化性胆管炎(PSC)是一种慢性胆汁淤积性肝病,其特征为胆道的慢性炎症和进行性纤维化。大多数 PSC 患者伴有炎症性肠病(IBD),这被认为可促进疾病的发展和进展。然而,肠道炎症可能加重胆汁淤积性肝病的分子机制仍不完全清楚。在这里,我们采用 IBD-PSC 小鼠模型来研究结肠炎对胆汁酸代谢和胆汁淤积性肝损伤的影响。出乎意料的是,在慢性结肠炎模型中,肠道炎症和屏障损伤可改善急性胆汁淤积性肝损伤并导致肝纤维化减少。这种表型与结肠炎引起的微生物胆汁酸代谢改变无关,但通过 LPS 诱导的肝细胞 NF-κB 激活介导,LPS 在体外和体内均抑制胆汁酸代谢。本研究确定了一种结肠炎触发的保护性回路,可抑制胆汁淤积性肝病,并鼓励对 PSC 采用多器官治疗策略。