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Updated: Apr 4, 2026

Complete Laparoscopic Radical Resection of Perihilar Cholangiocarcinoma Type IIIb

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Published on: January 17, 2025

Primary biliary cirrhosis.

Elizabeth J Carey¹, Ahmad H Ali¹, Keith D Lindor²

¹Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, AZ, USA.

Lancet (London, England)

|September 15, 2015

Summary

This summary is machine-generated.

Primary biliary cirrhosis (PBC) is a chronic liver disease. While ursodeoxycholic acid (UDCA) improves survival, new therapies are needed for non-responders, with nuclear receptor hormones showing promise.

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Area of Science:

Hepatology
Immunology
Gastroenterology

Background:

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by autoimmune destruction of small intrahepatic bile ducts.
Antimitochondrial antibodies (AMAs) are a specific serological marker, present in ~95% of PBC patients.
Fatigue and pruritus are common symptoms, independent of disease severity.

Purpose of the Study:

To review the current understanding of PBC epidemiology, pathogenesis, and natural history.
To discuss current management strategies and complications of PBC.
To introduce emerging therapeutic options, including research on nuclear receptor hormones.

Main Methods:

Review of existing literature on primary biliary cirrhosis.
Analysis of clinical trial data for ursodeoxycholic acid (UDCA) efficacy.
Exploration of research findings on novel therapeutic targets.

Main Results:

Ursodeoxycholic acid (UDCA) has improved transplant-free survival in PBC patients.
Approximately 40% of PBC patients exhibit an inadequate biochemical response to UDCA.
Liver transplantation offers excellent outcomes for patients with decompensated cirrhosis.

Conclusions:

Despite UDCA's benefits, a significant proportion of PBC patients require alternative treatments.
Research into nuclear receptor hormones presents promising avenues for new PBC therapies.
A comprehensive understanding of PBC's multifaceted aspects is crucial for advancing patient care.