What is primary biliary cholangitis (PBC)?

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease that harms the liver’s ability to function. It is chronic, which means it lasts for a long time or regularly comes back.

In people with PBC, the bile ducts become injured, then inflamed, and eventually permanently damaged. The bile ducts are small tubes in the liver that carry bile (a substance needed to digest food) from the liver to other parts of the digestive system.

When the bile ducts don’t work, bile builds up in the liver. This buildup can cause scarring in the liver (cirrhosis). Cirrhosis makes it hard for the liver to function properly. PBC is progressive, meaning it gets worse over time. If it is not treated, cirrhosis can cause liver failure and even death.

Healthy LiverLiver with Cirrhosis

Who is affected by primary biliary cholangitis (PBC)?

Doctors typically diagnose PBC in people between 35 and 60 years of age. Over 90 percent of patients who develop PBC are women. It affects people of all races. PBC is estimated to occur in about 65 of every 100,000 women and in 12 of every 100,000 men.

What causes primary biliary cholangitis (PBC)?

Doctors do not know for certain what causes PBC. Some experts believe it may be related to a problem with the immune system, where the immune system attacks healthy cells in the body. In PBC, doctors believe the immune system damages the bile ducts.

While there is not a known cause, some people with PBC also have other related autoimmune diseases. These conditions include autoimmune hepatitis, thyroid diseases, scleroderma, Raynaud’s diseases, Sjogren’s syndrome and celiac disease. Frequent urinary tract infections are also often seen in patients with PBC. In addition, infections, smoking and exposure to certain chemicals may play a role in triggering PBC.

PBC also tends to occur more commonly among family members. If one member of a family has PBC, the other family members are at an increased risk.

What are the symptoms of primary biliary cholangitis (PBC)?

Many people with PBC have no symptoms in the early stages. Some people discover they have the condition when a doctor tests them for another issue. As the condition progresses, affected people may first experience symptoms including:

  • Tiredness (fatigue)
  • Itchy skin (pruritus)
  • Abdominal pain
  • Darkening of the skin
  • Small yellow or white bumps under the skin or around the eyes
  • Dry eyes and mouth
  • Muscle and joint pain

In later stages of PBC, additional symptoms include:

  • Yellowing of the skin and whites of the eyes (jaundice)
  • Swelling of the legs, ankle and feet (edema)
  • Swelling of the abdomen from fluid buildup (ascites)
  • Internal bleeding in the upper stomach/lower esophagus from enlarged veins (varices)
  • Nausea
  • Weight loss
  • Dark-colored urine

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy