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Primary Sclerosing Cholangitis (PSC)
Overview
- What is PSC?
Primary sclerosing cholangitis (PSC) is rare, chronic liver disease that causes scarring and inflammation in the bile ducts of the liver. Bile is a liquid made by the liver that helps the body to digest fat. Bile ducts are the pipes that carry bile from the liver to the intestine. When bile ducts become damaged or blocked, bile cannot flow properly. Inflammation and scarring of the liver occur. This scarring can lead to serious liver damage or cirrhosis.
- What causes PSC?
The cause of PSC is not known. Studies suggest that problems with the immune system or long-term infections may be the cause. Although it appears possible, it is not known whether PSC can be inherited or passed down from other family members.
- How common is PSC?
PSC is rare. Seventy percent of patients with PSC are men. The average age at diagnosis is forty years. Seventy to eighty percent of people with PSC also have a type of inflammatory bowel disease. Three out of four of these people have ulcerative colitis, and the rest have Crohn’s disease. On the other hand, only about 4% of people with inflammatory bowel disease have PSC. /p>
Symptoms
- What are the symptoms of PSC?
Most people with PSC have no symptoms at all. However, some people notice tiredness, itching or diarrhea. Other symptoms can include fevers, chills, jaundice (yellow eyes and skin) and abdominal pain. Some people have a slow form of the disease, and they do not develop symptoms or problems for many years. Other people have a faster form of the disease, and serious problems like blocked bile ducts, liver damage, and liver failure happen early.
Diagnosis
- What tests are needed to evaluate PSC?
Blood Tests
Blood tests are often the first step. Liver tests should be checked. A liver test called alkaline phosphatase (ALP) is often high, but some patients may have normal levels. Other liver tests including the aminotransferases (ALT and AST) can also be abnormal. Bilirubin is usually normal early in the disease but may rise as the bile ducts become blocked. A significant rise in the alkaline phosphatase and bilirubin can indicate narrowing or blockage in the main bile duct. Albumin and prothrombin time two tests that show how well the liver is working. If these tests are abnormal, it means the liver is getting worse.
Cholangiogram
In the past, doctors used a procedure called cholangiography to diagnose PSC. A cholangiogram is an X-ray test that looks at the bile ducts using dye. Now, doctors usually use an imaging test called Magnetic Resonance Cholangiopancreatography (MRCP), which is a type of Magnetic Resonance Imaging (MRI). MRI is a medical imaging technique that uses magnetic fields and radio waves to produce detailed images of the body’s internal structures.
Cholangiography can still be helpful to treat patients with PSC when large bile ducts are blocked or if doctors think there might be cancer in the bile ducts, but it is no longer the main test used to diagnose PSC. Cholangiography is often done using a procedure called Endoscopic Retrograde Cholangiopancreatography (ERCP). In ERCP, a thin, flexible tube is passed through the mouth, down the throat, through the stomach, and into the first part of the small intestine. There is a small opening there that connects to the bile duct. Doctors can pass a tiny tube through this opening, inject dye, and take X-ray pictures to look for narrow areas or blockages.
If a patient cannot have ERCP, doctors may do another test called Percutaneous Transhepatic Cholangiography (PTC). In this test, a needle is placed through the skin into the liver, and dye is injected into the bile ducts before taking X-ray pictures.
Both of these tests are done with medicine to help the patient stay comfortable.
Magnetic Resonance Imaging
MRCP is now the main test used to diagnose PSC. This is a special type of MRI scan that takes pictures of the bile ducts without using tubes or needles. It is safe and painless. MRCP is usually the first imaging test to check for PSC. Studies show that MRCP is about 90-95% accurate in finding PSC and in ruling it out.
Liver Biopsy
A liver biopsy takes a tiny piece of liver tissue using a needle. The skin is numbed and a needle is inserted, typically between the ribs on the right side of the abdomen, into the liver. The tissue is then examined under a microscope. A liver biopsy can be helpful to diagnose PSC, see how much scarring is present, or judge the severity of scarring and liver damage.
- What are the complications of PSC?
People with PSC can develop other health problems:
- Dominant Stricture: Some people may develop a bile duct blockage. Bile is blocked from flowing out of the liver. This can cause yellow skin or eyes, infections, or bile duct stones. Doctors can do an ERCP and place a small tube called a “stent” to open the blockage.
- Fat Soluble Vitamin Deficiencies: Bile helps the body to absorb vitamins A, D, E and K, known as fat-soluble vitamins. If bile does not reach the intestines, fat-soluble vitamins may not be absorbed, and vitamin deficiencies can occur. People may have diarrhea or poor fat digestion.
- Osteoporosis: PSC can cause osteoporosis or bone thinning. This is diagnosed with a bone density scan (also called a dual-energy x-ray absortiometry, or DEXA scan) and may be treated with calcium or vitamin D supplements, or other medicines.
- Cholangiocarcinoma: People with PSC have a higher risk of developing bile duct cancer, called cholangiocarcinoma. Cholangiocarcinoma can occur in 7 to 15% of patients with PSC. Patients with a long history of inflammatory bowel disease and smokers are at the highest risk. This cancer can be hard to find early because symptoms of cholangiocarcinoma can be similar to symptoms of PSC. Patients with PSC should have regular MRCP procedures to screen for cholangiocarcinoma.
- Cirrhosis: Cirrhosis is severe scarring of the liver. This scarring can lead to liver failure. Patients with PSC and cirrhosis can develop serious problems such as bleeding from swollen veins in the esophagus, fluid buildup in the belly and legs, and confusion caused by toxins building up in the bloodstream. Cirrhosis is a common cause of death in the United States. It can be caused by many different conditions, such as viral hepatitis, or heavy alcohol use. To learn more about cirrhosis and its potential complications, please click here to access a patient brochure published by the ACG on cirrhosis.
- Colon Cancer: People with PSC have a higher risk of colon cancer. If you have PSC and inflammatory bowel disease, colonoscopy with biopsies throughout the colon once a year is recommended, starting at the time the PSC is diagnosis. Patients with PSC and inflammatory bowel disease also have a higher risk of colon cancer and colonoscopy every 5 years is recommended.
Treatment
- What are the treatments for PSC?
There is no cure for PSC. Medicines like ursodeoxycholic acid, colchicine, steroids, and methotrexate have been used to treat PSC but they are not effective. Treatment focuses on helping with symptoms and preventing complications. Patients with PSC should avoid things that can harm the liver, like alcohol. They should also get vaccines for hepatitis A and B. Patients with more advanced disease may need vitamin supplements.
Some medicines like diphenhydramine, cholestyramine, rifampin and naltrexone can help relieve itching. Osteoporosis is treated with calcium and vitamin D, and medications to strengthen the bones.
- When should liver transplantation be considered?
Patients who develop complications of cirrhosis and those with recurrent cholangitis are candidates for liver transplantation. Patients with PSC who undergo a liver transplant have very good survival rates and most experience a big improvement in their quality of life.
Author(s) and Publication Date(s)
Young-Mee Lee, MD, and Marvin Jose Lopez, MD, New England Medical Center, Boston, MA – Published December 2004. Updated January 2010.
Nader A. Dbouk, MD, Emory Transplant Center, Atlanta, GA and Ann D. Flynn MD, FACG, University of Utah, Salt Lake City, UT– Updated April 2026.