Cirrhosis, Primary Biliary
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- PBC primarily affects women; female:male ratio of 8 to 10:1 (1,2)
- Peak age: 40 to 60 years; rare in children (2)
- Rare, present in all parts of the world and all races and nationalities; more common in Northern European countries and in the Northern United States
- Annual incidence: 0.7 to 49/1,000,000
- Prevalence: 4 to 14/100,000 (2)
Etiology and Pathophysiology
- PBC is caused by a T-cell–mediated attack on biliary epithelial cells primarily small, intrahepatic bile ducts.
- Destruction causes cholestasis, which can lead to cirrhosis and liver failure.
- Bile duct destruction leads to retained bile acids and foamy hepatocyte degeneration (2).
- PBC primarily occurs in genetically susceptible people exposed to specific environmental triggers (1,2,3).
- Some studies have suggested that infection (bacterial and viral) may be a trigger.
- Clusters of cases suggest a possible role of a range of environmental toxins.
- Smoking increases risk of progression of fibrosis.
- PBC risk is increased following pregnancy-related pruritus or cholestasis in genetically susceptible individuals (1).
Commonly Associated Conditions
- PBC associated with: Sjögren syndrome, scleroderma, rheumatoid arthritis, lupus, autoimmune thyroiditis, Raynaud phenomenon, sarcoidosis, pulmonary fibrosis, polymyositis, celiac disease, and ulcerative colitis (2)
- 53% have one or more concurrent autoimmune conditions (1,2).
- Overlap syndrome with autoimmune hepatitis in 5–19%. Diagnosis is difficult and prognosis worse (1).
- Cirrhotic PBC increases relative risk of hepatocellular carcinoma (HCC). HCC occurs 1–6% of patients with PBC per year (1).