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Sex-related factors in autoimmune liver diseases

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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.

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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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Authors and Affiliations

  1. I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany

    Dorothee Schwinge & Christoph Schramm

  2. Martin Zeitz Centre for Rare Diseases, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, Hamburg, 20246, Germany

    Christoph Schramm

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  1. Dorothee Schwinge
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Correspondence to Dorothee Schwinge or Christoph Schramm.

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This article is a contribution to the special issue on Sex differences in immunity – Guest Editors: Hanna Lotter and Marcus Altfeld

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Schwinge, D., Schramm, C. Sex-related factors in autoimmune liver diseases. Semin Immunopathol 41, 165–175 (2019). https://doi.org/10.1007/s00281-018-0715-8

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