What is PBC? Discover the details behind this autoimmune disease
What to know about this rare liver condition
Actor portrayal.
On this page, we’ll find out what primary biliary cholangitis (PBC) is, what researchers believe causes it, learn who is at risk of developing it, how it can progress over time, and how it usually gets diagnosed.
IN THIS ARTICLE:
PBC BASICS
What is PBC?
PBC stands for “primary biliary cholangitis.” It is an autoimmune disease of the liver that causes inflammation.
PBC occurs when the immune system attacks bile ducts in the liver. Bile acids leak from the bile ducts into nearby liver tissue, causing tissue damage and additional immune responses.
This damage to the bile ducts is irreversible and so it’s important to seek treatment for PBC as soon as possible.
PBC can cause symptoms such as:
- Itching
- Fatigue
- “Brain fog” (forgetfulness, difficulty concentrating)
- Dry mouth or eyes
- Stomach (abdominal) pain
- Loss of bone density or strength
Left untreated, PBC can cause serious liver damage and permanent scarring, also known as cirrhosis. Cirrhosis could lead to liver failure and the need for a liver transplant.
PBC can destroy bile ducts in the liver, so early diagnosis and treatment are key.
What causes PBC?
Even though the exact cause of PBC is unknown, researchers have found some factors that are associated with a PBC diagnosis.
You are more likely to have PBC because of the genes handed down to you by your family. You could also have one or more environmental triggers such as:
- Urinary tract infections
- Reproductive hormone replacement
- Viruses or bacterial infections
- Exposure to certain chemicals
The exact role of lifestyle and daily habits in PBC is unclear, but it does not appear to be related to alcohol use.
With autoimmune diseases, the immune system, which normally protects you from things like bacteria and viruses, mistakenly begins to attack your own body.
Researchers believe there may be certain factors such as a person’s environment and their genes that can lead to PBC.
PATIENT DEMOGRAPHICS
Who gets PBC?
Even though there are over 130,000 people living with PBC in the US, the disease can seem hidden because many people don’t show symptoms at diagnosis.
For the most part, people tend to be diagnosed between 40 and 60 years of age.
Data show that 90% of people living with PBC are women. However, it is important to recognize that PBC can affect men and women from all ethnic and racial groups.
PBC may affect anyone. A common misperception is that PBC is a disease of White, middle-aged women. This is most likely due to diagnosis being delayed in other gender, racial, or ethnic groups.
Therefore, being aware of PBC symptoms is important for a timely diagnosis when talking to your doctor—especially for people who may not fit into the “typical” demographic profile.
People living with PBC often manage a number of other chronic conditions, such as:
- Bone density loss (osteoporosis)
- High cholesterol (hyperlipidemia)
- Cancer of the liver known as hepatocellular carcinoma
It’s important to discuss any other conditions you have when considering treatment options with your healthcare team.
SIGNS AND SYMPTOMS
What are common PBC symptoms?
PBC can cause symptoms such as itching, fatigue, brain fog, dry mouth or eyes, stomach (abdominal) pain, and others. Having these symptoms does not necessarily mean your disease is more advanced or severe.
Here’s a little more detail about some common symptoms:
Itching, also called “pruritus”
PBC itch can be very different from “typical” itching. For some, it feels like insects crawling under their skin or a burning sensation. Others describe an overwhelming urge to scratch so intense that it leads to bleeding or scarring. These are just a few of the ways people with PBC experience pruritus. The intensity of PBC itch can change over time, coming and going in “fits” or “episodes” and varying significantly from person to person.
Fatigue
PBC fatigue is more than just feeling tired. This chronic fatigue can impact your mental health, your overall well-being, and your ability to work and take care of daily chores. PBC itch can wake people up at night, interrupting sleep and adding to their fatigue.
Brain fog
People with PBC have reported feelings of brain fog when describing PBC fatigue. These feelings of confusion or slowed thinking can be connected to symptoms affecting brain function.
Quality of life
All or some of these symptoms can have a negative effect on quality of life. This might look like trouble sleeping, difficulty concentrating, or being too tired to go to work or do other daily activities. Talk to your healthcare team if you notice a change in your day-to-day routine.
I was experiencing symptoms like itching on the bottoms of my feet, my hands…It happened at night mostly…which led to fatigue.
Jackeline Candelario, living with PBC
Person featured was compensated by Gilead.
diagnosis and staging
How is PBC diagnosed?
PBC is usually diagnosed with blood tests that check for elevated liver enzymes, such as alkaline phosphatase (ALP), and the presence of antimitochondrial antibodies (AMA), which are found in 95% of PBC cases.
Other tools like imaging scans or liver biopsies may be used to look for liver damage and identify what stage the disease is in.
Once a PBC diagnosis has been found, there are key lab results and scans that are used to track the disease and see how you’re responding to treatment.
You may have the following blood tests or scans ordered by your healthcare team:
Alkaline phosphatase (ALP)
An enzyme (protein) released from stressed or injured bile duct cells and the main indicator for PBC.
Alanine aminotransferase (ALT)
An enzyme produced in the liver and found in the blood if you have liver disease.
Antimitochondrial antibody (AMA)
An autoantibody (an antibody created by misdirected immune responses) that can be present in people living with PBC.
Bilirubin (Bili)
A breakdown of red blood cells, normally cleared by the liver.
Ultrasound
A test that uses sound waves to look at tissues and organs inside the body.
FibroScan (transient elastography)
A special ultrasound that measures the stiffness (scar tissue) of your liver.
Once your doctor has reviewed these liver lab indicators and made a PBC diagnosis, this information can help them identify the stage of PBC you are in.
Learn more about ALP levels and what they mean
What are the stages of PBC and what do they mean?
Knowing the 4 stages of PBC is important to understanding disease progression and may inform appropriate treatment approaches.
Stages of PBC
STAGE 1
Inflammation in the bile ducts and some bile duct damage. However, liver function may still be relatively normal
STAGE 2
Increased inflammation, damage to the bile ducts (which can lead to more noticeable symptoms), and initial signs of liver fibrosis (scarring)
STAGE 3
Liver scarring has spread to other parts of the liver, impacting liver function
STAGE 4
Widespread scarring of liver tissue or cirrhosis. The liver no longer functions normally and there’s a much higher risk of liver failure or other complications, including liver cancer
The earlier treatment is started for PBC, the better the chance of preventing liver damage.
It’s recommended that people living with PBC have their liver labs checked every 3 to 6 months to see if the disease has worsened. The information provided is not meant to replace your treatment plan or advice from your healthcare team.