Primary Sclerosing Cholangitis

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Definition & Facts

In this section:

What is PSC?

Primary sclerosing cholangitis (PSC) is a chronic 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 liver damage. This damage can lead to cirrhosis and eventually liver failure. Medical experts believe that PSC is an autoimmune disease, which means that the immune system is overactive and attacks normal, healthy bile duct cells.

How common is PSC?

Researchers estimate that about 6 to 16 people out of 100,000 have PSC.1 However, this estimate may be low because not all people who have PSC are diagnosed with the disease.2

Who is more likely to get PSC?

You are more likely to get PSC if you

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.3

What are the complications of PSC?

Complications of PSC include

People with PSC and IBD have a greater chance of getting colorectal cancer.

References

Symptoms & Causes

What are the symptoms of PSC?

The main symptoms of primary sclerosing cholangitis (PSC) are

  • feeling tired or weak
  • itchy skin

Other symptoms may include

  • losing weight without trying
  • poor appetite
  • fever
  • pain in the abdomen

As the disease gets worse, you may get symptoms of cirrhosis and liver failure, such as

  • bloating
  • bruising and bleeding easily
  • confusion, difficulty thinking, or memory loss
  • redness in the palms of your hands
  • swelling in your legs, ankles, or feet
  • yellowish eyes and skin, called jaundice

Because PSC gets worse slowly, you can have the disease for years before you have any symptoms. Many people have no symptoms when they are first diagnosed with PSC.

Man scratching itchy skin on his arm.
One of the main symptoms of PSC is itchy skin.

What causes PSC?

The cause of PSC is not known. However, one or more of the following may play a role in causing the disease:

Diagnosis

How do doctors diagnose PSC?

Doctors diagnose primary sclerosing cholangitis (PSC) based on your medical and family history, a physical exam, and the results of medical tests.

Your doctor will ask you about your symptoms. He or she may also ask whether

During a physical exam, your doctor may

  • use a stethoscope to listen to sounds in your abdomen
  • tap or press on specific areas of your abdomen
  • look for symptoms of cirrhosis and liver failure
  • check to see if your liver and spleen are larger than they should be
  • check for tenderness or pain in your abdomen

Your doctor may also check for and ask about symptoms of a bile duct infection, which may include

  • fever
  • chills
  • nausea
  • vomiting
  • yellowish eyes and skin, called jaundice

What medical tests do doctors use to diagnose PSC?

Blood tests

Liver function tests can show abnormal liver enzyme levels in your blood. Abnormal levels of certain liver enzymes may be a sign of damage to your liver or bile ducts. Blood tests can also show higher-than-normal levels of certain antibodies in the blood, which may be a sign of PSC. Another blood test, called a white blood cell (WBC) count, measures the number of WBCs in your blood. A high WBC count may be a sign of a bile duct infection.

Red blood in test tube on a paper with test results.
Liver function tests can show abnormal liver enzyme levels in your blood.

Imaging tests

Your doctor may perform one or more of the following imaging tests of your liver and bile ducts:

Imaging tests may help diagnose PSC by ruling out other causes of bile duct damage, such as autoimmune hepatitis and tumors.

Liver biopsy

In some cases, your doctor may perform a liver biopsy to

  • confirm the diagnosis of PSC
  • see whether the disease is advanced, as shown by the amount of liver scarring or cirrhosis
  • rule out other diseases that may be causing your symptoms

Treatment

How do doctors treat PSC?

Doctors can’t cure primary sclerosing cholangitis (PSC) or keep the disease from getting worse. However, they can treat narrowed or blocked bile ducts and the symptoms and complications of PSC.

Narrowed or blocked bile ducts

If your bile ducts are narrowed or blocked, your doctor may use medical procedures, such as endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography, to open them and help keep them open.

Itchy skin

Your doctor may recommend over-the-counter products and medicines or prescribe medicines to treat itchy skin. Over-the-counter products and medicines include

For mild itchy skin, your doctor may prescribe hydroxyzine. For severe itchy skin, your doctor may prescribe cholestyramine.

Low levels of fat-soluble vitamins in your body

If you have low levels of fat-soluble vitamins in your body, your doctor may recommend dietary supplements of vitamins A, D, E, and K. Follow your doctor’s instructions on the type and amount of vitamins you should take.

Bile duct infection

Your doctor may prescribe an antibiotic to treat a bile duct infection.

Cirrhosis

If your PSC has caused cirrhosis, your doctor may treat the health problems related to cirrhosis with medicines, surgery, and other medical procedures. If cirrhosis leads to liver failure, you may need a liver transplant.

Prescription bottle on its side with pills.
Your doctor may prescribe antibiotics to treat a bile duct infection.

When do doctors consider a liver transplant for PSC?

Your doctor may consider a liver transplant if your PSC has caused liver failure. Doctors consider liver transplants only after all other treatment options have failed.

What can I do to prevent further liver damage?

To help prevent further liver damage, you can do the following:

  • Carefully follow your doctor’s instructions.
  • Take your medicines and vitamins as directed.
  • If you smoke, quit smoking.
  • Do not drink any alcohol or use illegal drugs.
  • Have regular checkups, as recommended by your doctor.
  • Talk with your doctor before taking
  • Try to keep a healthy body weight.

Eating, Diet, & Nutrition

What should I eat if I have PSC?

You should eat well-balanced meals that give you enough calories and nutrients. If you have low levels of fat-soluble vitamins in your body, your doctor may recommend eating foods that are high in vitamins A, D, E, and K. A health care professional such as a dietitian or nutritionist can help you plan meals that are good sources of these vitamins.

Grilled fish and vegetables on a plate.
If you have PSC, you should eat well-balanced meals that give you enough calories and nutrients.

What should I avoid eating if I have PSC?

You should avoid eating raw shellfish such as oysters, which can have bacteria that may cause severe infections in people with liver disease. Your doctor may recommend that you avoid foods that are high in salt, fat, and carbohydrates, especially those with added sugars.

Your doctor will recommend that you stop drinking alcohol, or at least limit your intake to no more than one or two drinks per week. If you have PSC and cirrhosis, your doctor will recommend that you don’t drink any alcohol at all.

Clinical Trials

The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and other components of the National Institutes of Health (NIH) conduct and support research into many diseases and conditions.

What are clinical trials and are they right for you?

Clinical trials are part of clinical research and at the heart of all medical advances. Clinical trials look at new ways to prevent, detect, or treat disease. Researchers also use clinical trials to look at other aspects of care, such as improving the quality of life for people with chronic illnesses. Find out if clinical trials are right for you.

Watch a video of NIDDK Director Dr. Griffin P. Rodgers explaining the importance of participating in clinical trials.

What clinical trials are open?

Clinical trials that are currently open and are recruiting can be viewed at www.ClinicalTrials.gov.

January 2018
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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. The 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 the NIDDK is carefully reviewed by NIDDK scientists and other experts.

The NIDDK would like to thank:
Keith D. Lindor, M.D., Arizona State University