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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
Review

Abstract

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.

Keywords

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

Abbreviations

AIH

Autoimmune hepatitis

PBC

Primary biliary cholangitis

PSC

Primary sclerosing cholangitis

AILD

Autoimmune liver diseases

LT

Liver transplantation

ANA

Antinuclear antibody

ASMA

Anti-smooth muscle antibody

LKM 1-anti

Liver kidney microsome 1 antibody

anti-LC-1

Anti-liver cytosol type 1 antibodies

AMA

Anti-mitochondrial antibody

PDC-E2

Pyruvate dehydrogenase complex

UDCA

Ursodeoxycholic acid

OVA

Obeticholic acid

IBD

Inflammatory bowel disease

UC

Ulcerative colitis

AP

Alkaline phosphatase

ALT

Alanine aminotransferase

gGT

Gamma-glutamyl transpetidase

HCC

Hepatocellular carcinoma

ER

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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