Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is the most common autoimmune liver disease. PBC is characterized as a chronic inflammatory autoimmune cholestatic liver disease where immune-mediated injury to biliary epithelial cells leads to cholestasis and fibrosis, and if left untreated, PBC will progress to end-stage liver disease. The disease affects predominantly middle-aged women, with data suggesting 1 in 1000 women over the age of 40 live with PBC. Importantly, clinical presentation ranges from being completely asymptomatic to significantly affecting the patient’s quality of life via pruritus, fatigue, abdominal pain, and sicca symptoms (dry mouth, dry eyes) in addition to liver-related complications. In this chapter, we describe the classic presentation of an asymptomatic patient with a cholestatic pattern of liver injury. We discuss the initial work-up and differential diagnosis for this pattern, the diagnostic criteria for PBC, and specific treatment, with an emphasis on early referral to a liver specialist to ensure the proper management of this progressive liver disease.
KeywordsCholestasis Treatment Ursodeoxycholic acid Obeticholic acid Risk stratification Pruritus
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