Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, 20246, Germany.
Martin Zeitz Center for Rare Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, 20246, Germany.
Semin Immunopathol. 2022 Jul;44(4):485-507. doi: 10.1007/s00281-022-00936-6. Epub 2022 May 10.
Autoimmune liver diseases are a group of immune-mediated liver diseases with three distinct entities, including autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis. The interplay of genetic and environmental factors leads to the breakdown of self-tolerance, resulting in hyper-responsiveness, and auto-aggressive immune activation. Emerging evidence links autoimmune liver diseases with alterations of the commensal microbiome configuration and aberrant immune system activation by microbial signals, mainly via the gut-liver axis. Thus, the microbiome is a new frontier to deepen the pathogenetic understanding, uncover biomarkers, and inspire innovative treatments. Herein, we review the current evidence on the role of the microbiome in autoimmune liver diseases from both clinical and basic research. We highlight recent achievements and also bottlenecks and limitations. Moreover, we give an outlook on future developments and potential for clinical applications.
自身免疫性肝病是一组免疫介导的肝脏疾病，具有三种不同的实体，包括自身免疫性肝炎、原发性胆汁性胆管炎和原发性硬化性胆管炎。遗传和环境因素的相互作用导致自身耐受的破坏，导致过度反应和自身攻击性免疫激活。新出现的证据将自身免疫性肝病与共生微生物组结构的改变和微生物信号引起的异常免疫系统激活联系起来，主要通过肠道-肝脏轴。因此，微生物组是深化发病机制理解、发现生物标志物和激发创新治疗方法的新前沿。在此，我们从临床和基础研究两个方面综述了微生物组在自身免疫性肝病中的作用的现有证据。我们强调了最近的成就，以及瓶颈和局限性。此外，我们还展望了未来的发展方向和临床应用的潜力。
