4.5 Review

The intestinal and biliary microbiome in autoimmune liver disease-current evidence and concepts

Journal

SEMINARS IN IMMUNOPATHOLOGY
Volume 44, Issue 4, Pages 485-507

Publisher

SPRINGER HEIDELBERG
DOI: 10.1007/s00281-022-00936-6

Keywords

Intestinal barrier; Liver disease; Microbiome; Microbiota; Autoimmunity; Autoimmune hepatitis; Primary biliary cholangitis; Primary sclerosing cholangitis

Funding

  1. German Research Foundation (DFG) Clinical Research Unit 306 Primary sclerosing cholangitis [KFO306]
  2. Landesforschungsforderung Hamburg [FV-78]
  3. YAEL Foundation
  4. German Research Foundation [420943353, 438122637]

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Autoimmune liver diseases are a group of immune-mediated liver diseases that are associated with abnormalities in the gut microbiome and immune system activation. Current research has made progress in understanding the role of the microbiome, but there are still limitations and challenges. Future studies and clinical applications hold potential for further advancements.
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.

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