PBC Basics

The prognosis of primary biliary cholangitis (PBC) varies from patient to patient. While in some, the disease progresses slowly over many years, others have more aggressive forms of the disease, and they progress to liver failure much faster. However, the average time it takes for PBC to progress to the terminal stage is around 15 to 20 years.  

Stages of PBC

PBC is divided into four stages. If left untreated, the disease progresses from one stage to the next in an average of one and a half to two years.

Stage one, also called the portal stage, is associated with bile duct changes and/or portal inflammation. Stage twi is characterized by periportal fibrosis and/or inflammation with dilated portal tracts. Stage three is the septal stage in which septal fibrosis is present with active inflammation and/or paucicellular septa. Finally, stage four is the cirrhosis stage, in which variously sized nodules and varying degrees of inflammation are apparent.

Factors affecting prognosis

Factors that are associated with faster progression and worse outcomes include:

• Young age at diagnosis. 
• Male gender. 
• Presence of cirrhosis.
• High levels of bilirubin and alkaline phosphatase in the serum.
• Low serum levels of albumin.
• Esophageal varices.
• Presence of hepatocellular carcinoma.
• Lack of response to treatment with ursodeoxycholic acid (UDCA). 

Racial differences and associated healthcare disparities can also affect disease prognosis in PBC, with Black and Hispanic patients having a significantly higher mortality rate compared to White patients. Indigenous Canadian patients are more commonly diagnosed at later stages of the disease and have lower event-free and transplant-free survival compared to White patients.

It is not known whether the presence of symptoms at the time of diagnosis affects the prognosis of the disease. Treatment with UDCA and liver transplantation can significantly improve prognosis and disease outcome.

Survival rate 

Research has shown that the five-, ten- and 15-year survival rates of patients with PBC who have not been treated are 79%, 59% and 32%, respectively.

On the contrary, for patients who have been treated with UDCA, the five-, ten- and 15-year survival rates are much higher, at 90%, 78% and 66%, respectively. 

The Mayo Risk Score, an approved mathematical model, can help predict survival in individual patients with PBC who have not had a liver transplant.

For patients with PBC who had a successful liver transplant, the 10-year survival rate is 65%.

Lifestyle changes that could improve prognosis

PBC is a chronic disease that progresses over many years. Here are some lifestyle changes that may improve the prognosis of the disease. Please note that these changes should always be made under the supervision of a healthcare professional.

• Maintain a healthy lifestyle (eat a healthy diet and limit smoking and drinking).
• Be physically active.
• Reduce stress.
• Always consult with your doctor before taking any supplements or herbal remedies, as these may be harmful to your liver.

Life expectancy

Once the symptoms of the disease appear, the average life expectancy of patients with PBC is around 10 years.

Reviewed by Harshi Dhingra, M.D., on December 18, 2023.