PBC is a progressive and chronic disease, but immediate treatment may delay the damage it causes

Although primary biliary cholangitis (PBC) affects each person differently, certain characteristics are common in each stage and phase of the disease’s progression.a A liver biopsy is required to see what stage of PBC you are in.

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

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
  • Liver biopsies are not necessary to diagnose PBC, so you and your doctor may decide to avoid one
  • Healthcare teams will use the most advanced stage they see to categorize the stage of the disease

aIt 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.

PBC may still be uncontrolled—even on treatment.

  • For many people, the first medicine their doctor prescribes is not enough to adequately control their PBC
  • It’s important to discuss with your doctor how you’re responding to treatment
  • Ask about additional treatment options if your PBC is still uncontrolled
“The consultant didn’t force me to have the biopsy, he sort of wanted me to. But it was still my choice.” — Wendy, England

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

  • Alkaline phosphatase (ALP)

    Elevated levels of ALP indicate damage to the liver

  • Bilirubin

    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 tracks 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
  • If your ALP is still too high, your doctor may consider adding an additional medicine to help get your level under control

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
  • If your ALP level remains high on your current treatment, ask about a different medicine that may help lower it

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.

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All participating parties, including patients, physicians, and experts, were compensated by ICPT.

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