Abstract
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.
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Scheinberg, A.R., Levy, C. (2019). Primary Biliary Cholangitis. In: Cohen, S., Davitkov, P. (eds) Liver Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-98506-0_17
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DOI: https://doi.org/10.1007/978-3-319-98506-0_17
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