Definition & Facts for Primary Sclerosing Cholangitis

In this section:

What is PSC?

Primary sclerosing cholangitis (PSC) is a chronic liver disease in which the bile ducts inside and outside the liver become inflamed and scarred, and are eventually narrowed or blocked. When the bile ducts are narrowed or blocked, bile builds up in the liver and causes further liver damage. This damage can lead to cirrhosis and, eventually, liver failure. Medical experts believe PSC is an autoimmune disease, in which the immune system attacks normal, healthy bile duct cells.

How common is PSC?

Researchers estimate about 5 to 16 people out of every 100,000 have PSC.1

Who is more likely to develop PSC?

PSC is more commonly diagnosed in people who1,2

Female doctor talking with a male patient.PSC affects twice as many males as females.

What other health problems do people with PSC have?

People with PSC may have other health problems, including

About 7 out of 10 people who have PSC also have IBD.1

What are the complications of PSC?

PSC can lead to liver complications such as cirrhosis, cancers, and bile duct infection.

Liver complications

PSC can damage the liver, leading to cirrhosis and its complications.


In cirrhosis, scar tissue replaces healthy liver tissue and prevents your liver from working normally. As cirrhosis gets worse, the liver begins to fail.

Portal hypertension

Portal hypertension most often occurs when scar tissue in the liver slows the normal flow of blood, which causes high blood pressure in the portal vein. The portal vein is the large blood vessel that carries blood from your stomach, intestines, spleen, gallbladder, and pancreas to the liver.

When portal hypertension reaches a certain level, it can cause additional complications, such as

Liver failure

Cirrhosis may eventually lead to liver failure, also called end-stage liver disease. With liver failure, your liver is badly damaged and stops working. People with liver failure may require a liver transplant.


PSC can increase the chance of developing certain cancers.

  • Bile duct cancer is the most common type of cancer in people who have PSC. People with PSC have a 10% to 20% chance of developing bile duct cancer at some point in their lives.3
  • People with PSC have an increased chance of getting gallbladder cancer.
  • People with cirrhosis due to PSC have an increased chance of getting liver cancer.
  • People with PSC and IBD have an increased chance of getting colorectal cancer.

Bile duct infection

People with PSC may develop a bacterial infection in narrowed or blocked bile ducts. Medical procedures that affect the bile ducts, such as endoscopic retrograde cholangiopancreatography, increase the chance of bile duct infection.

Other complications

Other complications of PSC may include


Last Reviewed February 2022
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This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health. NIDDK translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by NIDDK is carefully reviewed by NIDDK scientists and other experts.