Gail Cawkwell, MD, PhD, Senior Vice President, Medical Affairs at Intercept, provides an overview of primary biliary cholangitis (PBC), a rare liver disease that is caused by an autoimmune reaction. The autoimmune reaction damages bile ducts in the liver. Bile ducts are tube-like structures that carry bile from the liver to the intestine to help with the digestion of food.
When bile is not able to move through the bile ducts, it collects in the liver and causes damage. PBC is progressive, which means that the damage gets worse over time. Starting with inflammation, the damage can cause fibrosis, and then cholangitis. In some cases, cholangitis can lead to liver failure.
It is estimated that 90% of people who are diagnosed with PBC are women. Many people who are diagnosed with PBC are between 40-60 years of age. It is important to note, however, that men and young women can also develop PBC.
The most common symptoms of PBC are pruritus, or itching, and fatigue. Some people with PBC can also experience dry eyes and mouth. For people with more advanced PBC, serious liver damage can cause jaundice, or a yellowing of the skin and eyes. Many people do not experience any symptoms at the beginning of their PBC. The only sign that they have PBC is that abnormalities appear in their liver function test results.
Doctors can test for PBC by taking blood samples and measuring certain chemicals related to liver function. The tests will show if there are elevated levels of alkaline phosphatase (ALP) and if antimitochondrial antibodies (AMAs) are present, which can be indicators of PBC.