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Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis (primary biliary cirrhosis)

Raoul Poupon, MD
Section Editor
Keith D Lindor, MD
Deputy Editor
Kristen M Robson, MD, MBA, FACG


Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance (picture 1). The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis and eventually may result in cirrhosis and liver failure [1-3]. The terminology was changed from primary biliary cirrhosis to primary biliary cholangitis to more accurately describe the disorder and its natural history [4]. With the advent of treatment with ursodeoxycholic acid, the majority of patients now have normal life expectancies and only a minority of patients develops cirrhosis. (See "Pathogenesis of primary biliary cholangitis (primary biliary cirrhosis)".)

This topic will review the clinical manifestations and diagnosis of PBC. It will also review factors associated with prognosis. The pathogenesis of PBC, the treatment of PBC, and the treatment of pruritus due to cholestasis are discussed separately. (See "Pathogenesis of primary biliary cholangitis (primary biliary cirrhosis)" and "Overview of the treatment of primary biliary cholangitis (primary biliary cirrhosis)" and "Liver transplantation in primary biliary cholangitis (primary biliary cirrhosis)" and "Pruritus associated with cholestasis".)

The American Association for the Study of Liver Diseases issued a practice guideline for PBC in 2009 [5]. The discussion that follows is generally consistent with that guideline.


Primary biliary cholangitis (PBC) is rare, with a reported prevalence of 19 to 402 cases per million persons [6,7]. The vast majority of patients (90 to 95 percent) are women, and most patients are diagnosed between the ages of 30 and 65 years (often in their 40s or 50s), though the disease has been reported in women as young as 15 years and as old as 93 years [8-10].

The incidence of PBC developing in a well-defined population from Rochester, Minnesota was estimated to be 2.7 per 100,000 person-years (4.5 per 100,000 person-years for women and 0.7 per 100,000 person-years for men) [6]. The age and gender-adjusted prevalences per 100,000 persons were 65.4 for women and 12.1 for men.

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Literature review current through: Dec 2017. | This topic last updated: Jan 11, 2017.
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