Seminars in Immunopathology

, Volume 41, Issue 2, pp 165–175 | Cite as

Sex-related factors in autoimmune liver diseases

  • Dorothee SchwingeEmail author
  • Christoph SchrammEmail author


Autoimmune diseases are a broad range of diseases in which the immune system produces an inappropriate response to self-antigens. This results in inflammation, damage, or dysfunction of tissues and/or organs. Many autoimmune diseases are more common in women and differences between female and male immune and autoimmune responses have been well documented. In general, most of the autoimmune diseases seem to affect more females, although there are exceptions. Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are considered to be autoimmune liver diseases (AILD). They all are rare diseases and they result in significant morbidity and mortality. The female predominance in PBC and AIH are among the strongest among autoimmune diseases. However, the mechanisms responsible for the sex differences in autoimmune liver diseases are largely unknown. In this review, we discuss the recent findings on the influence of sex-dependent mechanisms, which may contribute to differences in presentation, clinical characteristics, disease course, and complications observed between female and male patients with autoimmune liver disease.


Autoimmune hepatitis (AIH) Primary biliary cholangitis (PBC) Primary sclerosing Cholangitis Pregnancy Testosterone Estradiol X Chromosome Microbiome 



Autoimmune hepatitis


Primary biliary cholangitis


Primary sclerosing cholangitis


Autoimmune liver diseases


Liver transplantation


Antinuclear antibody


Anti-smooth muscle antibody

LKM 1-anti

Liver kidney microsome 1 antibody


Anti-liver cytosol type 1 antibodies


Anti-mitochondrial antibody


Pyruvate dehydrogenase complex


Ursodeoxycholic acid


Obeticholic acid


Inflammatory bowel disease


Ulcerative colitis


Alkaline phosphatase


Alanine aminotransferase


Gamma-glutamyl transpetidase


Hepatocellular carcinoma


Estrogen receptor


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.I. Department of MedicineUniversity Medical Center Hamburg-EppendorfHamburgGermany
  2. 2.Martin Zeitz Centre for Rare DiseasesUniversity Medical Center Hamburg-EppendorfHamburgGermany

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