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Primary biliary cirrhosis: from induction to destruction.
A Nishio1, N M Bass, V A Luketic
1Department of Gastroenterology, Tenri Hospital, Nara, Japan.
Seminars in Gastrointestinal Disease
|May 16, 2001
Summary
Primary biliary cirrhosis (PBC) is a chronic liver disease affecting middle-aged women, characterized by bile duct destruction and antimitochondrial antibodies. Ursodeoxycholic acid is the main therapy, delaying liver transplantation but not curing the disease.
Area of Science:
- Hepatology
- Immunology
- Gastroenterology
Background:
- Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease.
- It predominantly affects middle-aged women, with fatigue and pruritus as common symptoms.
Observation:
- Liver function tests show elevated alkaline phosphatase and gamma-glutamyl transpeptidase.
- Histology reveals intrahepatic small bile duct destruction and fibrosis.
- Antimitochondrial antibodies, targeting 2-oxo-acid dehydrogenase complexes, are present in 95% of patients.
Findings:
- PBC progresses slowly, potentially leading to liver failure.
- Symptomatic patients with advanced age, elevated bilirubin, low albumin, or cirrhosis have shortened survival.
- Current immunosuppressive and anti-inflammatory drugs lack curative effects.
Implications:
- Ursodeoxycholic acid is the only effective therapy to delay liver transplantation and improve survival.
- Some patients on ursodeoxycholic acid still progress, necessitating transplantation.
- Liver transplantation remains the definitive treatment for end-stage PBC.