Primary sclerosing cholangitis (PSC) is a chronic inflammatory progressive cholestatic liver disease. Genetic risk factors, presence of autoantibodies, the strong clinical link with inflammatory bowel disease (IBD), and associations with other autoimmune disorders all suggest a pivotal role for the immune system in PSC pathogenesis. In this review, we provide a comprehensive overview of recent immunobiology insights in PSC. A particular emphasis is given to immunological concepts such as tissue-residency and knowledge gained from novel technologies including single-cell RNA sequencing (scRNA-seq) and spatial transcriptomics (ST). This review of the immunobiological landscape of PSC covers major immune cell types known to be enriched in PSC-diseased livers as well as recently described cell types whose biliary localization and contribution to PSC immunopathogenesis remain incompletely described. Finally, we emphasize the importance of time and space in relation to PSC heterogeneity as a key consideration for future studies interrogating the role of the immune system in PSC.

中文翻译：

---

原发性硬化性胆管炎的免疫生物学


原发性硬化性胆管炎（PSC）是一种慢性炎症性进行性胆汁淤积性肝病。遗传危险因素、自身抗体的存在、与炎症性肠病 (IBD) 的密切临床联系以及与其他自身免疫性疾病的关联都表明免疫系统在 PSC 发病机制中发挥着关键作用。在这篇综述中，我们全面概述了 PSC 的最新免疫生物学见解。特别强调免疫学概念，例如组织驻留和从单细胞 RNA 测序 (scRNA-seq) 和空间转录组学 (ST) 等新技术中获得的知识。本篇对 PSC 免疫生物学概况的综述涵盖了已知在 PSC 患病肝脏中富集的主要免疫细胞类型，以及最近描述的其胆道定位和对 PSC 免疫发病机制的贡献仍未完全描述的细胞类型。最后，我们强调时间和空间对于 PSC 异质性的重要性，作为未来研究免疫系统在 PSC 中的作用的关键考虑因素。