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Hypercholesterolemia in primary biliary cholangitis (primary biliary cirrhosis)

Steven Flamm, MD
Andre A Kaplan, MD
Raoul Poupon, MD
Sanjiv Chopra, MD, MACP
Section Editor
Keith D Lindor, MD
Deputy Editor
Kristen M Robson, MD, MBA, FACG


Hypercholesterolemia is a common feature of primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) and other forms of cholestatic liver disease. The mechanism of hyperlipidemia in cholestatic disorders is different from that in other conditions because unusual lipoprotein particles, such as lipoprotein-X, may accumulate and levels of high-density lipoprotein cholesterol are typically elevated.

Hypercholesterolemia (ie, cholesterol values above 200 mg/dL [5.2 mmol/L]) affects approximately 75 percent of patients with primary biliary cholangitis at presentation, but is not associated with an increased risk of atherosclerosis [1-3]. However, patients with primary biliary cholangitis may have other independent risk factors for cardiovascular disease that require attention in the setting of normal life expectancy with ursodeoxycholic acid treatment.

This topic will review hypercholesterolemia in patients with primary biliary cholangitis. The pathogenesis and management of hypercholesterolemia and its role in the development of atherosclerosis are discussed separately.

(See "Screening for lipid disorders in adults".)

(See "Overview of established risk factors for cardiovascular disease".)

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Literature review current through: Nov 2017. | This topic last updated: Dec 04, 2017.
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