Hypercholesterolemia in primary biliary cholangitis (primary biliary cirrhosis)
- Steven Flamm, MD
Steven Flamm, MD
- Chief, Liver Transplantation Program
- Professor of Medicine
- Feinberg School of Medicine
- Northwestern University
- Andre A Kaplan, MD
Andre A Kaplan, MD
- Professor of Medicine
- University of Connecticut Health Center
- Raoul Poupon, MD
Raoul Poupon, MD
- Professor of Hepatology and Gastroenterology
- University Pierre et Marie Curie
- UPMC, Sorbonne University, Paris, France
- Sanjiv Chopra, MD, MACP
Sanjiv Chopra, MD, MACP
- Editor-in-Chief — Gastroenterology and Hepatology
- Section Editor — General Hepatology; Gallbladder and Biliary Tract Disease
- Professor of Medicine
- Harvard Medical School
- Senior Consultant in Hepatology
- James Tullis Firm Chief
- Beth Israel Deaconess Medical Center
INTRODUCTION AND PREVALENCE
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.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- INTRODUCTION AND PREVALENCE
- CHOLESTEROL METABOLISM IN PBC
- Low-density lipoprotein receptors
- CLINICAL FEATURES
- Signs and symptoms
- Laboratory findings
- RISK OF CARDIOVASCULAR DISEASE
- Clinical and laboratory monitoring
- Candidates for lipid lowering agents
- - Use of statins or fibrates
- Treatment for xanthomas
- SUMMARY AND RECOMMENDATIONS