Primary biliary cholangitisa (PBC) is a progressive and chronic disease, but immediate treatment may delay the damage it causes.

Although PBC affects each person differently, certain characteristics are common in each stage and phase of the disease's progression.b A liver biopsy is required to see what stage of PBC you are in.

Stage 1
  • Inflammation of the bile ducts in the liver
  • Some people may test positive for antimitochondrial antibodies (AMAs)
  • Normal liver function test results
  • No symptomsb
Stage 2
  • Continued inflammation that may cause scarring in the bile ducts
  • AMA positive
  • Abnormal liver function test results (ALP may rise)
  • No symptoms
Stage 3
  • Additional scarring in the liver
  • Abnormal liver function test results (increased ALP levels; and bilirubin may rise)
  • Symptoms such as fatigue (tiredness) and/or pruritus (itching) may first appear
Stage 4
  • Advanced scarring in the liver, including cirrhosis
  • Abnormal liver function test results (ALP levels stay elevated; and bilirubin is elevated)
  • Symptoms of cirrhosis such as jaundice may appear

But PBC does not affect all parts of the liver equally.

  • A single liver sample taken in a liver biopsy may show signs of several stages at once, and it may not give the doctor enough information for an accurate determination of disease stage
  • Healthcare teams will use the most advanced stage they see to categorize the stage of the disease
  • Liver biopsies are no longer necessary to diagnose PBC, and you and your doctor may decide to avoid a liver biopsy and not determine the stage of your PBC

a Formerly known as primary biliary cirrhosis.
b It is important to understand that symptoms such as fatigue and pruritus are not necessarily related to how far PBC has progressed. For some people, those symptoms may even become less severe as their livers become more damaged.

A simple blood test can measure 2 key markers that are important for monitoring your PBC treatment and liver health:


Alkaline phosphatase (ALP)

(elevated levels of ALP indicate damage to the liver)



(especially elevated in the later stages of the disease)

Serious problems linked with PBC can include the following:

Vitamin deficiency, where the body has trouble absorbing important vitamins

Fluid retention and swelling in the stomach, called ascites

Osteoporosis, a type of bone disease that causes weakening of the bones

Bleeding in the esophagus, stomach, or intestines, called varices, caused by an increase in pressure known as portal hypertension

A kind of liver cancer known as hepatocellular carcinoma

Eventually, people with PBC can progress to liver failure and may need a liver transplant

The best way to slow the progression of your PBC is to take the medicine your healthcare provider prescribes and to make sure it is working for you.


Medical guidelines recommend that your healthcare team track your ALP level by checking it every 3 to 6 months.

  • They can use the results to see how well the medicine is working to lower your ALP

To make sure you are keeping up with your ALP tests, it can be helpful to:

  • Set a reminder in your phone or on your calendar to get your ALP level checked every 3 to 6 months
  • After each ALP test, ask your healthcare team to set up your next appointment
  • Track your ALP test results on your smartphone using the PBC Living® app

Without treatment, the rate of progression of PBC varies—the time it takes for the disease to cause damage is different for each person.


It is important to note that for most people, PBC progresses slowly from one phase to the next. People can have the disease for a decade or more before they reach cirrhosis.

In people who do not treat their PBC, the disease may progress from one stage to the next as fast as every 1.5 years.

  • PBC may progress more slowly if it is caught and treated early
  • Some people who are diagnosed and treated early have a lower chance of experiencing liver failure or liver transplant than those who are not treated early

You can take action.

Ask your healthcare provider to test your ALP level every 3 to 6 months so you can track your results.