| [ Summary ] |
Primary biliary cholangitis (PBC) is a liver‒specific autoimmune disease that is characterized by a preponderance of female cases, the presence of disease‒specific anti‒mitochondrial antibodies, and the histological destruction of intrahepatic bile ducts. PBC eventually leads to cirrhosis and hepatic failure. In the clinical setting, patients exhibiting cholestasis along with anti‒mitochondrial antibody positivity require differential diagnosis of PBC. The number of identified asymptomatic PBC cases has recently increased due to wider recognition and earlier detection of the disease, with the vast majority showing a favorable response to ursodeoxycholic acid therapy. Nonetheless, some patients experience progression to cirrhosis, hepatic failure, and in rare cases hepatocellular carcinoma. Since several factors have been associated with a diminished prognosis, it is important to accurately diagnose the clinical stage of PBC. Therefore, simple and reliable non‒invasive biomarkers to estimate liver fibrosis and PBC progression are needed. In this section, two major points are summarized: 1) the epidemiology and diagnosis of PBC, and 2）non‒invasive biomarkers to estimate disease status. |