Primary biliary cholangitis

Primary biliary cholangitis (PBC) is characterized by progressive intrahepatic bile duct damage and loss.

The prevalence of PBC is up to 35/100,000 in US populations, with a distribution that is heavily skewed toward women (up to 10:1 female-to-male distribution) and those over 45 years of age.

The combination of a cholestatic pattern of serum liver tests (elevated alkaline phosphatase, gamma-glutamyl transferase, or both) and a PBC-specific autoantibody (typically antimitochondrial antibody) is sufficient for diagnosis in most patients, with no need for biopsy confirmation.

PBC is progressive in most patients; although, in many people, the rate of progression can be so slow that 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.

Obeticholic acid is recommended in patients with an inadequate response to ursodiol.

Transplantation is an effective treatment for those patients who develop end-stage liver disease with PBC.

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 (classically antimitochondrial antibodies) has led to the widely held view that PBC is an autoimmune disease.[1]Jones DE. Pathogenesis of primary biliary cirrhosis. Gut. 2007;56:1615-1624. http://www.ncbi.nlm.nih.gov/pubmed/17641080?tool=bestpractice.com