Sex-related factors in autoimmune liver diseases

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.