The prevalence of primary biliary cholangitis (PBC) is up to 35/100,000 in US populations, with a distribution that is heavily skewed toward women (10:1 female-to-male distribution) and those over 45 years of age.
PBC is progressive in most patients, although in many people the rate of progression can be so slow it may not be clinically relevant. Cirrhosis and its typical complications arise in the end stage.
Symptoms (typically pruritus and fatigue) can significantly lower the quality of life, even in patients with a very slowly progressive disease. These symptoms warrant treatment in their own right using specific regimens.
Progression of the disease can be slowed by therapy with ursodiol.
Transplantation is an effective treatment for those patients who develop end-stage liver disease with PBC.
Obeticholic acid should be used in patients showing an inadequate response to ursodiol.
Primary biliary cholangitis (PBC) is a chronic disease of the small intrahepatic bile ducts that is characterized by progressive bile duct damage (and eventual loss) occurring in the context of chronic portal tract inflammation. Fibrosis develops as a consequence of the original insult and the secondary effects of toxic bile acids retained in the liver, resulting ultimately in cirrhosis. The almost universal presence of autoantibodies in PBC patients (classically antimitochondrial antibodies) has led to the widely held view that the disease has an autoimmune component to its etiology. 
Professor of Liver Immunology
University of Newcastle
Honorary Consultant Hepatologist
DEJJ has received speaker honoraria from Falk, grant funding from Intercept and Pfizer, and has undertaken consultancy work for Falk, GSK, Intercept, and Novartis. DEJJ is an author of a number of articles referenced in this monograph.
Queen Elizabeth Hospital
JN declares that he has no competing interests.
Department of Biochemistry and Molecular Biology
IRM declares that he has no competing interests.
Professor of Medicine
Chief of Clinical Gastroenterology and Hepatology
University of Texas Medical Branch
NS declares that he has no competing interests.
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