It is a chronic liver disease, where the body destroys the cells lining the bile ducts in the liver. They are called the intrahepatic bile ducts. If left untreated it can lead to scarring of the liver, which can cause liver failure.
Symptoms include abdominal pain, fatigue, itchiness and jaundice of the skin. Jaundice occurs when PBC is severe as the liver is so damaged that it's normal function is impaired. Many people with PBC have no symptoms and it is discovered due to abnormal results on routine liver blood tests.
It has been estimated that PBC is one of the most common autoimmune diseases, affecting nearly 1 in 1000 women over the age of 40. NORD
Diagnosis of PBC requires the presence of:
- High alkaline phosphatase (ALP, a liver blood test) along with
- Positive anti-mitochondrial antibody (+AMA)
It is thought that PBC is most common in Northern Europe and North America, although it is found in people all over the world. PBC Foundation