Primary biliary cholangitis (PBC) used to be called primary biliary cirrhosis and is a type of liver disease caused by damage to the bile ducts in the liver. Much like other forms of liver disease, PBC permanently damages the liver as tissue is replaced with scar tissue (fibrosis). As more scar tissue develops, the structure and function of the liver are affected.
What causes PBC?
The exact cause of PBC is unknown. But it may be related to a problem with the immune system, which is the body's natural defense system.
What are the symptoms?
Many people who have primary biliary cholangitis do not have symptoms. When symptoms do occur, they may include:
Yellowing of the skin and the white part of the eyes (jaundice).
Discomfort in the upper right part of the abdomen.
Dry eyes and mouth.
With more advanced liver damage, people may have complications related to cholangitis such as:
Fluid buildup in the abdomen (ascites).
Bleeding of enlarged veins in the esophagus, stomach, and rectum (variceal bleeding).
Premature thinning of the bones (osteoporosis).
How is PBC diagnosed?
Primary biliary cholangitis is suspected based on a medical history, physical exam, and lab tests that show high levels of the enzyme alkaline phosphatase and antimitochondrial antibodies (AMA) in the blood. Your doctor may also conduct an imaging test such as an ultrasound, which shows images of the liver and other tissues inside the body. He or she may also do a biopsy to remove a small piece of tissue from the liver. Biopsy is the best way to definitively diagnose PBC.
How is it treated?
Treatment for primary biliary cholangitis is difficult, because the immune system appears to be involved. As with other forms of liver disease, treatment focuses on reducing symptoms, preventing and treating the complications of the disease, and preventing other conditions that may cause additional liver damage.
Two medicines, ursodiol (UDCA) and Ocaliva, can be used to treat PBC. Both medicines help move bile out of the liver into the small intestine. And Ocaliva decreases the amount of bile acids made by the liver.
Medicines such as cholestyramine, rifampicin, or naltrexone may be used to help with itching caused by PBC.
If you have fatigue, your doctor will try to find the cause of your tiredness. It may not be caused by PBC. Some medicines have been tried to help with fatigue in PBC, but none have been proved to work well.
PBC can also cause dry eyes and mouth. The best way to help dry eyes is to use eye drops (artificial tears) when your eyes feel dry. A dry mouth can be helped by chewing gum or hard candy to increase saliva. You can also use a saliva substitute. If that doesn't help, there are some medicines that can be used.
Because people with PBC have a high risk of getting osteoporosis, you should have periodic bone density scans. Your doctor may suggest that you take calcium and vitamin D supplements and perhaps a medicine called a bisphosphonate.
Liver transplantation may be the treatment of choice for people who have end-stage primary biliary cholangitis, although primary biliary cholangitis can recur after a liver transplant.
Angulo P, Lindor KD (2010). Primary biliary cirrhosis. In M Feldman et al., eds., Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed., vol. 2, pp. 1477â€“1488. Philadelphia: Saunders.
Talwalkar JA, Lindor KD (2006). Primary biliary cirrhosis. In M Wolfe et al., eds., Therapy of Digestive Disorders, 2nd ed., pp. 579â€“587. Philadelphia: Saunders Elsevier.
ByHealthwise StaffPrimary Medical ReviewerAdam Husney, MD - Family MedicineE. Gregory Thompson, MD - Internal MedicineSpecialist Medical ReviewerW. Thomas London, MD - Internal Medicine, Hepatology