Highlights & Basics
- Primary biliary cholangitis (PBC) is characterized by progressive intrahepatic bile duct damage and loss.
- PBC is significantly more common in women than in men. Peak incidence is around age 40 years, and median age at diagnosis is 65 years.
- The combination of a cholestatic pattern of serum liver tests (elevated alkaline phosphatase, gamma-glutamyl transferase, or both) and a PBC-specific autoantibody (typically antimitochondrial antibody) is sufficient for diagnosis in most patients, with no need for biopsy confirmation.
- PBC is progressive in most patients; although, in many people, the rate of progression can be so slow that it may not be clinically relevant. Cirrhosis and its typical complications arise in the end stage.
- Symptoms (typically pruritus and fatigue) can significantly lower the quality of life, even in patients with a very slowly progressive disease. These symptoms warrant treatment in their own right using specific regimens.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Characteristic autoantibody patterns in primary biliary cholangitis. White arrow: antimitochondrial staining; red arrow: multiple nuclear dot ANA staining
Characteristic histologic appearances of primary biliary cholangitis: (a) early-stage disease; (b) advanced-stage disease; (c) disease with a significant inflammatory component
Citations
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