A disease that starts suddenly and without a clear cause
Related to or affecting the bile ducts in the liver
Inflammation of the bile ducts
PBC is a chronic, progressive autoimmune liver condition that affects the bile ducts in the liver. A long-term treatment plan that includes medicine to help slow the progression of PBC can help prevent serious health issues, including the need for a liver transplant.
Your bile ducts play an important role in keeping your liver healthy. With PBC:
Your body begins attacking your bile ducts, causing a buildup of toxic bile acid (cholestasis)
Cholestasis causes further inflammation and damage to the liver cells
Ongoing cell damage leads to harmful scarring (fibrosis), which can result in cirrhosis over time
Once you have cirrhosis, liver damage is mostly permanent and end-stage liver disease (transplant or death) is possible
You may have heard of cirrhosis caused by drinking alcohol, but the cirrhosis related to PBC is not due to drinking alcohol.
A PBC diagnosis can take a long time because it is a rare disease, so many healthcare providers may not check for it until they have ruled out more common conditions first. In fact, many people who have PBC get diagnosed because their liver shows signs of damage during routine liver testing.
Healthcare providers will look at some key markers for diagnosis:
Healthcare providers may also perform a liver biopsy to confirm a diagnosis of PBC and to find out the level of PBC progression. After diagnosis, noninvasive tests such as liver ultrasound or magnetic resonance imaging (MRI) scan can check if there is fibrosis.
How much itching or fatigue you feel is not related to how far PBC has progressed or how well your treatment is working.
Autoimmune conditions commonly associated with PBC include:
In some people, PBC can progress very quickly. Treatment may work best in people whose PBC is still in early stages. So, it’s important to speak to your healthcare team about starting the treatment that’s right for you and your unique needs.
When I attended my first meeting with the support group for PBC… that was the first time that I had ever seen anyone besides my father who had PBC.
—Wayne, living with PBC
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