What Is Primary Biliary Cholangitis?

Learning you have primary biliary cholangitis (PBC) is complicated. You may have questions about how it will affect you. Understanding the condition can help you feel more in control.

PBC is a rare liver condition. It happens when your immune system attacks healthy cells in the liver. It was previously called primary biliary cirrhosis; the name was changed because not everyone who has PBC has cirrhosis. Many people with PBC learn they have it from routine blood tests.^1,2

This article explains what causes PBC. It also covers how PBC progresses. You will learn about health outcomes with common treatments.

Causes and risk factors

PBC is an autoimmune condition. Autoimmune conditions happen when your immune system mistakenly attacks healthy cells.^1,2

The word “biliary” refers to your bile ducts. Bile ducts carry bile from your liver to other organs. Bile helps you digest fats and absorb vitamins. In PBC, your immune system attacks your bile ducts. Constant damage leads to inflammation called “cholangitis.”^1,2

The word “primary” means that the condition itself causes inflammation, rather than infections or alcohol. You did not do anything to cause PBC.³

Experts do not know what causes the autoimmune reaction. Genetics and environmental triggers play a role. These triggers do not cause PBC. But they may increase your risk for PBC. Risk factors include:^1,2,4

• People assigned female at birth
• Age between 30 to 60
• Having a family member with PBC
• Northern European ancestry
• Certain infections, such as urinary tract infections
• Smoking cigarettes
• Exposure to toxic chemicals

Who is affected by PBC?

Anyone can get PBC. It is most common in middle-aged women from Europe and North America. Rates of PBC diagnosis are rising, possibly because screening tests for liver function are becoming more common.^1,2

Women are 9 times as likely to have PBC as men and make up about 90 percent of PBC cases. Experts do not yet know the reasons for this. PBC affects about 1 in 1,600 women and 1 in 6,500 men in the US. Diagnosis is most common between ages 30 and 60.^1,2

Stages of PBC/PBC progression

PBC is a chronic and progressive condition. It does not go away. It can get worse over time. When bile ducts are destroyed, bile builds up in the liver. Over time, this can kill liver cells. Scar tissue then replaces healthy liver cells. This can make it hard for the liver to work properly.¹

If left untreated, PBC can lead to liver failure and death. Treatments slow or stop progression. PBC progresses through a few stages:^2,3,5

• Stage 1 – Small bile ducts in the liver become inflamed. You may not have symptoms or signs of liver damage in blood work.
• Stage 2 – Inflammation spreads to medium-sized bile ducts. You may not have symptoms. Bloodwork may show signs of liver damage.
• Stage 3 – Scar tissue builds in the liver. Symptoms start. Bloodwork shows signs of liver damage.
• Stage 4 – Liver scarring becomes severe. This is called cirrhosis. Serious complications can happen, including liver failure.

PBC prognosis, life expectancy, and outlook

There is no cure for PBC, but there are medications that can slow or stop disease progression. You may live for many years without PBC interfering with daily life. Fatigue is often the hardest symptom to control.⁶

Your health outlook depends on the stage of PBC. In most cases, PBC progresses slowly. It takes 15 to 20 years for PBC to progress to the final stage. Many people with PBC never develop cirrhosis, the most serious stage.⁶

When PBC is diagnosed earlier, life expectancy may be normal. The first stage can last a long time. Half of people start showing symptoms 5 to 10 years after diagnosis. Average life expectancy is about 10 years after symptoms start.^3,6

Progression to liver failure can happen and is most common for people who are diagnosed late. Higher levels of bilirubin in the blood predict faster progression. Bilirubin is a chemical that gives bile its yellow color.^2,5,6

People who progress to liver failure may need a liver transplant. Liver transplants have high success rates for people with PBC. For some people, PBC comes back after a liver transplant.^2,6