Biliary fibrosis is seen in cholangiopathies, including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). In PBC and PSC, biliary fibrosis is associated with worse outcomes and histologic scores. Within the liver, both hepatic stellate cells (HSCs) and portal fibroblasts (PFs) contribute to biliary fibrosis, but their roles can differ. PFs reside near the bile ducts and may be the first responders to biliary damage, whereas HSCs may be recruited later and initiate bridging fibrosis. Indeed, different models of biliary fibrosis can activate PFs and HSCs to varying degrees. The portal niche can be composed of cholangiocytes, HSCs, PFs, endothelial cells, and various immune cells, and interactions between these cell types drive biliary fibrosis. In this review, we discuss the mechanisms of biliary fibrosis and the roles of PFs and HSCs in this process. We will also evaluate cellular interactions and mechanisms that contribute to biliary fibrosis in different models and highlight future perspectives and potential therapeutics.

中文翻译：

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细胞相互作用和串扰促进胆汁淤积中的胆道纤维化

胆道纤维化见于胆管病，包括原发性胆汁性胆管炎（PBC）和原发性硬化性胆管炎（PSC）。在 PBC 和 PSC 中，胆道纤维化与较差的结果和组织学评分相关。在肝脏内，肝星状细胞 (HSC) 和门静脉成纤维细胞 (PF) 都会导致胆道纤维化，但它们的作用可能不同。 PF 位于胆管附近，可能是胆道损伤的第一反应者，而 HSC 可能稍后被招募并引发桥接纤维化。事实上，不同的胆道纤维化模型可以不同程度地激活 PF 和 HSC。门静脉生态位可由胆管细胞、HSC、PF、内皮细胞和各种免疫细胞组成，这些细胞类型之间的相互作用驱动胆道纤维化。在这篇综述中，我们讨论了胆道纤维化的机制以及 PF 和 HSC 在此过程中的作用。我们还将评估不同模型中导致胆道纤维化的细胞相互作用和机制，并强调未来的前景和潜在的治疗方法。