What is Primary Biliary Cholangitis (PBC)?
Formerly called primary biliary cirrhosis, this condition was recently renamed as primary biliary cholangitis or PBC. It is a rare condition that is more common in women than men and typically occurs in middle age.
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PBC is a rare, progressive disease of the small bile ducts inside of the liver. Bile is a liquid produced by the liver cells. When bile is unable to be properly drained due to damaged bile ducts inside of the liver, there is buildup of bile (cholestasis). Cholestasis leads to chronic inflammation of the liver and scarring. Over time, scarring is irreversible and can progress to cirrhosis. This can lead to liver failure and even liver cancer.
Classic symptoms of PBC include:
- Severe itching (pruritus)
- Jaundice (yellowing of the skin and eyes)
Some individuals may not initially have symptoms present, but rather may have abnormal liver chemistries (elevation in the alkaline phosphatase levels).
What Causes Primary Biliary Cholangitis?
The exact cause of PBC is not known, but researchers believe it is an autoimmune disease, meaning the body’s own immune system attacks the bile duct cells and destroys them. Others think that environmental factors may play a role in triggering the disease.
UH Offers Expert Diagnosis and Treatment of PBC
An accurate diagnosis of PBC is essential so that the appropriate treatments can be determined.
Diagnosis is made with blood tests and sometimes a liver biopsy.
There is no cure for PBC but once the diagnosis is confirmed, recommended treatments may include:
- Medications to help delay disease progression and ongoing monitoring
- Managing any complications that may arise from the disease
- Consideration for liver transplant in those who develop advanced disease
- Participation in a clinical trial