Cirrhosis, Primary Biliary
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Primary biliary cirrhosis (PBC) should be suspected in women with unexplained pruritus or cholestatic pattern of liver enzymes.
- PBC primarily affects women (95%) (2).
- Peak age: 40–60 years; can occur earlier, rarely in children
- Rare, but present universally; more common in northern European countries and in the northern US
- Prevalence ranges from 7–400 cases/million; dependent on varied diagnostic criteria, physician awareness, quality of health care, methods of detection, and case-finding.
Risk FactorsSmoking increases risk of disease development:
- Data also indicate that pregnancy may be a trigger for PBC; hormone replacement therapy is a possible, yet unproven precipitating factor.
- Diverse autoimmune diseases are associated with PBC.
- PBC is believed to result in individuals with susceptible genetic makeup (multiple inherited defects in immune tolerance) exposed to specific environmental triggers.
- Concordance rate is as high as 63% in monozygotic twins.
- Family predisposition: 1–6% of individuals with PBC have at least 1 family member with the disease.
- PBC is caused by a T-cell–mediated attack on intralobular bile ducts.
- T cells are targeted against mitochondrial antigens responsible for oxidative phosphorylation.
- The autoimmune attack is isolated to small, intrahepatic bile ducts for unknown reasons.
- This destruction causes cholestasis, which can lead to cirrhosis and liver failure.
- The retained bile acids from bile duct destruction leads to foamy degeneration of hepatocytes.
Commonly Associated Conditions
- PBC can be associated with diverse autoimmune diseases, including Sjögren syndrome, scleroderma, rheumatoid arthritis, lupus erythematosus, autoimmune thyroiditis, celiac disease, and ulcerative colitis (3)[A].
- Overlap syndrome with autoimmune hepatitis exists in 5–15%. Diagnosis may be difficult and prognosis worse
- Compared with the general population, PBC is associated with a relative risk of hepatocellular carcinoma as high as 18.8.