Primary Biliary Cirrhosis

Primary biliary cirrhosis (also called primary biliary cholangitis) is a chronic disease that slowly damages and destroys the bile ducts. This causes bile to build up in the liver, which can lead to progressive scarring and cirrhosis.

Key Facts

  • Primary biliary cirrhosis is considered an autoimmune disease, which means it is caused by the body’s own immune system mistakenly attacking itself.
  • Most patients do not experience any symptoms when diagnosed. But as damage to the liver becomes more severe, serious health complications can emerge.
  • While there is no cure, various treatments are available that can slow the progression of the disease, relieve symptoms, and prevent complications.


Although the causes of primary biliary cirrhosis are still not completely understood, it’s thought to be an autoimmune disease. This means that the white blood cells that make up the immune system attack the healthy cells in the bile ducts rather than germs and other foreign substances. A mix of genetic and environmental factors may cause this to happen.

Several factors are known to increase the likelihood of developing primary biliary cirrhosis:

  • Age: People between the ages of 30 and 60 are most likely to develop this condition.
  • Family History: Genetic factors may make it more likely to develop this condition if a family member has also had it.
  • Gender: Women are more likely than men to develop primary biliary cirrhosis.
  • Geography: Primary biliary cirrhosis is most often found in northern Europe and North America.


Because primary biliary cirrhosis develops slowly, most patients do not have any symptoms when diagnosed. It typically takes between five and 20 years to develop symptoms.

Early symptoms may include the following:

  • Darkening or discoloration of the skin
  • Dry eyes and mouth
  • Fatigue
  • Itchy skin

Later symptoms may include the following:

  • Abdominal pain
  • Fatty deposits around the eyes, eyelids, or in the creases of the palms, soles, elbows, or knees (xanthomas)
  • Fluid in the abdomen (ascites)
  • Greasy diarrhea
  • High cholesterol
  • Jaundice (yellowing of eyes and skin)
  • Memory problems
  • Swollen feet and ankles
  • Weight loss


Diagnosing primary biliary cirrhosis usually begins with a physical examination. This will involve checking for any signs of liver inflammation and asking about family medical history.

The following are some other common ways to diagnose primary biliary cirrhosis:

  • Blood Tests: These test for high levels of cholesterol and liver enzymes, as well as for antimitochondrial antibodies, which are common in people with primary biliary cirrhosis.
  • Imaging Tests: These form an image of the liver and bile ducts so that doctors can better detect signs of disease. These tests may include a computerized tomography (CT) scan, an ultrasound, or an MRI (magnetic resonance imaging) test.
  • Liver Biopsy: This is a surgical procedure that removes a tiny portion of liver tissue so that doctors can examine it under a microscope to determine what is wrong.


There is no cure for primary biliary cirrhosis. However, there are treatment options that can help reduce and manage its complications. The following are some of the most common treatments:

  • Medications: A variety of medications are available that can help improve liver function, reduce scarring and inflammation, move bile through the liver, and treat other symptoms. Ursodeoxycholic acid (also known as ursodiol) is commonly used first.
  • Liver Transplantation: In severe cases, a liver transplantation may be necessary. This involves surgically removing the diseased liver and replacing it with a healthy donor liver. In some cases, primary biliary cirrhosis can recur in the new liver. Learn more about liver transplantations.


Successfully managing the symptoms and complications of primary biliary cirrhosis will depend on multiple factors. These include whether or not symptoms are present at diagnosis, how well their liver is functioning, and how well they adhere to their treatment regimen.

Liver transplantations have been linked with good long-term outcomes for patients with primary biliary cirrhosis. However, making healthy lifestyle changes can also help reduce symptoms and prevent further complications. These include regularly exercising, following a healthy diet, and reducing alcohol consumption.

In general, the average survival rate for patients who do not have symptoms is 16 years, while the survival rate for patients who do have symptoms is 7.5 years.

Next Steps

Our liver specialists are well-versed in diagnosing primary biliary cirrhosis and helping patients manage symptoms and reduce complications. We are also a leader in liver transplantations.

Call us at (877) LIVER MD/ (877) 548-3763 or use our online form to schedule an appointment.