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Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy
Treta Purohit1, Mitchell S Cappell1
1Treta Purohit, Mitchell S Cappell, Division of Gastroenterology and Hepatology, William Beaumont Hospital, Royal Oak, MI 48073, United States.
World Journal of Hepatology
|May 9, 2015
Summary
Primary biliary cirrhosis (PBC) is a chronic liver disease affecting middle-aged women. Treatment involves ursodeoxycholic acid, with liver transplantation for advanced stages.
Area of Science:
- Hepatology
- Autoimmune Diseases
- Gastroenterology
Background:
- Primary biliary cirrhosis (PBC) is a chronic, autoimmune, cholestatic liver disease.
- Characterized by anti-mitochondrial antibodies (AMA), bile duct destruction, and cholestasis.
- A variant, PBC-autoimmune hepatitis (AIH) overlap, presents with additional autoimmune markers and piecemeal necrosis.
Purpose of the Study:
- To provide a comprehensive overview of Primary Biliary Cirrhosis (PBC).
- To detail the clinical features, diagnosis, and prognosis of PBC.
- To outline current and emerging therapeutic strategies for PBC and its complications.
Main Methods:
- Review of existing literature on Primary Biliary Cirrhosis.
- Analysis of diagnostic criteria, including serological markers and liver biopsy findings.
- Evaluation of prognostic scoring systems and treatment modalities.
Main Results:
- PBC typically affects middle-aged females and presents with fatigue, pruritus, and jaundice.
- Ursodeoxycholic acid is the primary treatment, with corticosteroids used for PBC-AIH overlap.
- Liver transplantation offers definitive treatment for advanced disease with good survival rates.
Conclusions:
- Early diagnosis and management of PBC are crucial for improving patient outcomes.
- Multifaceted treatment approaches are necessary to manage symptoms and prevent complications.
- Ongoing research into novel therapies like obeticholic acid and fibrates holds promise for PBC treatment.
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