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.
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
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 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.
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.
What causes PSC?
Experts aren’t sure what causes PSC. Studies suggest that several factors may play a role, including
signs that the liver and spleen are larger than they should be
tenderness or pain in the abdomen
What tests do doctors use to diagnose PSC?
Blood tests
Liver tests can show abnormal liver enzyme levels in your blood.
A health care professional will take a blood sample from you and send the sample to a lab.
Liver tests can show abnormal levels of liver enzymes and other substances in your blood. Abnormal levels of certain liver enzymes may be a sign your liver or bile ducts are damaged.
Imaging tests
To diagnose PSC, doctors typically order a special imaging test to examine the bile ducts, such as
magnetic resonance cholangiopancreatography (MRCP), which uses a magnetic resonance imaging (MRI) machine to create pictures of the bile ducts. MRCP is the most common test that doctors use to diagnose PSC.
percutaneous transhepatic cholangiography (PTC), which is an x-ray of the bile ducts. A health care professional inserts a needle through the skin and into the liver to inject a special dye into the bile ducts. The special dye lets a doctor see the bile ducts on the x-ray.
Doctors may also order other imaging tests to check for signs of PSC, other bile duct or liver problems, or complications. These tests include
ultrasound, which uses a hand-held device, called a transducer, that bounces safe, painless sound waves off organs to create an image of their structure.
computed tomography (CT) scans, which uses a combination of x-rays and computer technology to create images of the liver.
elastography, a special test that measures the stiffness of the liver. Increased liver stiffness may be a sign of fibrosis, or scarring.
Liver biopsy
A liver biopsy is generally not needed to diagnose PSC. However, in some cases, doctors may order a liver biopsy to check for signs of other liver diseases, such as autoimmune hepatitis.
During a liver biopsy, a doctor will take small pieces of tissue from the liver. A pathologist will examine the tissue under a microscope.
Colonoscopy
For people who have PSC and haven’t already been diagnosed with IBD, doctors may recommend a colonoscopy to check for IBD. Many people with PSC have mild IBD and don’t have IBD symptoms.
Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC.
Narrowed or blocked bile ducts
If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open.
Doctors may recommend over-the-counter medicines or prescribe medicines to treat itchy skin caused by PSC.
How do doctors treat the complications of PSC?
Liver complications
Doctors may recommend treatments for liver complications of PSC.
Cirrhosis or portal hypertension
If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery.
PSC increases the risk for developing several types of cancer. Your doctor may recommend tests to check for signs of cancer. Finding cancer at an early stage improves the chance of curing the cancer.
To treat a bile duct infection, doctors may prescribe antibiotics. To help prevent bile duct infections, doctors may prescribe antibiotics for people with PSC before and after they have procedures, such as ERCP, that increase the risk of getting an infection.
For low levels of vitamins A, D, E, and K, doctors may recommend taking dietary supplements of these fat-soluble vitamins. Follow your doctor’s instructions on the type and amount of vitamins you should take.
For osteoporosis, doctors may prescribe medicines that slow or stop bone loss and improve bone density. Doctors may recommend taking dietary supplements of calcium and vitamin D.
When do doctors consider a liver transplant for PSC?
Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. Talk with your doctor to find out whether a liver transplant is right for you.
Doctors may consider a liver transplant if PSC leads to liver failure.
What can I do to prevent further liver damage?
If you have PSC, you can take steps to help prevent further liver damage.
Follow your doctor’s instructions carefully and take medicines and dietary supplements as directed.
People with primary sclerosing cholangitis (PSC) should eat a healthy, well-balanced diet. Good nutrition is important in all stages of PSC—including cirrhosis—to help the liver work properly and manage complications.
Your doctor can recommend a healthy diet that provides enough calories and nutrients. Your doctor may recommend taking dietary supplements of calcium and vitamin D to help prevent osteoporosis. For low levels of fat-soluble vitamins A, D, E, or K, your doctor may recommend taking supplements of these vitamins. Follow your doctor’s instructions on the type and amount of vitamins you should take.
What should I avoid eating if I have PSC?
You should avoid eating raw or undercooked shellfish, fish, meat, and unpasteurized milk. Bacteria or viruses from these foods may cause severe infections in people with liver disease.
Doctors may recommend that people with PSC stop drinking alcohol or, at least, limit their intake. People who have PSC and cirrhosis should completely stop drinking alcohol.
Clinical Trials
The NIDDK conducts and supports clinical trials in many diseases and conditions, including liver diseases. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.
What are clinical trials for PSC?
Clinical trials—and other types of clinical studies—are part of medical research and involve people like you. When you volunteer to take part in a clinical study, you help doctors and researchers learn more about disease and improve health care for people in the future.
Researchers are conducting clinical studies to better understand liver diseases, such as primary sclerosing cholangitis (PSC). For example, researchers are studying
Watch a video of NIDDK Director Dr. Griffin P. Rodgers explaining the importance of participating in clinical trials.
What clinical studies for PSC are looking for participants?
You can view a filtered list of clinical studies on PSC that are federally funded, open, and recruiting at www.ClinicalTrials.gov. You can expand or narrow the list to include clinical studies from industry, universities, and individuals; however, the NIH does not review these studies and cannot ensure they are safe. Always talk with your health care provider before you participate in a clinical study.
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.
The NIDDK would like to thank: Christopher L. Bowlus, M.D., University of California Davis School of Medicine
PSC affects twice as many males as females.
