Primary Biliary Cholangitis - PBC

Primary biliary cholangitis (PBC), previously referred to as primary biliary cirrhosis, is an autoimmune liver disorder characterized by chronic, progressive cholestatic disease. If untreated, PBC leads to cirrhosis, or scarring of the entire liver, which can result in liver failure.    The prevalence of PBC is estimated at 19-402 per million individuals.   PBC is more common in women than in men,    and usually arises at 40-50 years of age. Symptoms of PBC may include chronic pruritus (itching), fatigue, sicca symptoms, abdominal pain, jaundice, and arthralgia.    PBC is thought to develop through a combination of genetic and environmental factors, including urinary tract infections, reproductive hormone replacement, nail polish use, and cigarette smoking.  Additional risk factors include the presence of another autoimmune disorder (particularly celiac disease, systemic sclerosis, thyroid disease, and Sjögren syndrome  ) and family history of PBC.    Laboratory tests in the workup of PBC include liver biochemistry tests (such as alkaline phosphatase [ALP] and aminotransferase tests) and tests for autoantibodies (including antimitochondrial M2 antibodies [AMAs] and antinuclear antibodies [ANAs] such as anti-sp100 and antiglycoprotein 210 [anti-gp210] antibodies). Liver biochemistry tests can be used for prognosis and to monitor treatment response.   

Tabs Content
Content Review: 
April 2019

Last Update: April 2019