Definition & Facts for Primary Sclerosing Cholangitis

In this section:

• What is PSC?
• How common is PSC?
• Who is more likely to develop PSC?
• What other health problems do people with PSC have?
• What are the complications of PSC?

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

• are between the ages of 30 and 40, although PSC may occur at any age.
• are male. PSC affects twice as many males as females.
• have inflammatory bowel disease (IBD), most commonly ulcerative colitis.

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

• IBD
• gallstones
• autoimmune hepatitis
• other autoimmune diseases, such as type 1 diabetes, celiac disease, and thyroid diseases

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.

Cirrhosis

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

• swelling in the legs, ankles, or feet, called edema
• buildup of fluid in the abdomen, called ascites
• enlarged veins—called varices—in the esophagus, stomach, or intestines, which can lead to gastrointestinal bleeding if the veins burst
• confusion or difficulty thinking caused by a buildup of toxins in the brain, called hepatic encephalopathy

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.

Cancer

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

• low levels of fat-soluble vitamins A, D, E, and K
• osteoporosis, or loss of calcium from the bones

References

[1] Tabibian JH, Bowlus CL. Primary sclerosing cholangitis: a review and update. Liver Research. 2017;1(4):221–230. doi:10.1016/j.livres.2017.12.002

[2] Tabibian JH, Ali AH, Lindor KD. Primary sclerosing cholangitis, part 1: epidemiology, etiopathogenesis, clinical features, and treatment. Gastroenterology & Hepatology (NY). 2018;14(5):293–304.

[3] Rabiee A, Silveira MG. Primary sclerosing cholangitis. Translational Gastroenterology and Hepatology. 2021;6:29. Published April 5, 2021. doi:10.21037/tgh-20-266

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