Primary Sclerosing Cholangitis (PSC)

In primary sclerosing cholangitis (PSC), the bile ducts (a type of passageway) inside and outside the liver become narrowed due to inflammation and scarring. As the scarring increases, the ducts become narrowed or blocked. The ducts are important because they carry bile out of the liver.

Bile is a liquid that removes toxins from the body and helps break down fat in food. If the ducts are narrowed or blocked, toxins in bile remain in the liver and damage liver cells. In addition, fat and vitamins A, D, E and K may not be well absorbed. Eventually, primary sclerosing cholangitis can cause liver failure.

Approximately one out of every 100,000 individuals in the world is diagnosed with primary sclerosing cholangitis.

Current research suggests that the cause of primary sclerosing cholangitis is immune mediated.  When the immune system is working correctly, it protects the body from infections caused by bacteria and viruses.

In the case of immune mediated disease, the body’s immune system causes an inflammatory response against itself. In the case of PSC, the inflammation causes restriction or blockage of bile ducts.

In many cases, primary sclerosing cholangitis occurs along with a type of inflammatory bowel disease, ulcerative colitis, in which the colon becomes inflamed and develops ulcers. It can also occur with autoimmune hepatitis or Crohn’s disease.

Since primary sclerosing cholangitis progresses slowly, a person can have the disease for years before symptoms develop. Symptoms are caused by two things: the bile is not being drained properly through the bile ducts and the liver is damaged and not able to carry out its usual functions.

The symptoms of liver disease and bile duct damage are:

  • Itching (may occur when toxins in bile get into the bloodstream) 
  • Fatigue (feeling tired all the time) 
  • Jaundice (causes yellowing of the eyes or skin; the color change is due to bile that gets in the blood and eventually in the skin and eyes) 
  • Fever, chills and soreness in the upper part of the stomach (caused by infected bile ducts)

As the disease progresses, chronic fatigue, loss of appetite, weight loss and continued jaundice may occur. In the advanced stages, swelling can occur in the stomach and feet. Liver failure may take many years to develop.

Since people with primary sclerosing cholangitis may not have noticeable symptoms for many years, the disease often is suspected due to abnormal blood tests (taken for other reasons) that show a high level of liver enzymes (which indicate abnormal liver function). The disease also might be suspected due to a history of inflammatory bowel disease.

X-ray techniques can reveal whether bile ducts are blocked; however, they may not be able to determine the cause or site of the possible obstruction.

Sclerosing cholangitis is diagnosed by blood tests, along with radiologic imaging.  Cholangiography is “taking a picture of the bile ducts.” The first choice for imaging is by injecting dye into the bile ducts and taking a magnetic resonance cholangiography (MRCP). The test can determine the cause and site of the blockage.

Many times a liver biopsy is also needed to confirm diagnosis and determine the progression of the disease.

Other tests that can be used to help with the diagnosis are:

  • Endoscopic retrograde cholangiography (ERCP) in which dye is injected and a lighted and flexible endoscope (instrument used to visually examine the inside of certain body parts) is inserted through the mouth, stomach and then into the small intestine. A thin tube is placed through the scope and into the pancreatic and bile ducts, and the dye is injected to show the bile ducts on the X-ray.
  • Percutaneous transhepatic cholangiography is when a dilated (enlarged) bile duct is found with an ultrasound, a needle punctures the skin and places dye directly into the bile duct and then X-rays are taken to identify blockage.

All of these procedures are done with the child sedated (given a medication that has a soothing and calming effect) or with anesthesia (medication given to put the child in a deep sleep with support to breathing available.)

Currently there is no cure for primary sclerosing cholangitis. Treatment is directed at managing symptoms and opening narrowed bile ducts. Symptoms of this disease can sometimes be managed by: 

  • Antibiotics to treat bile ducts that have become infected 
  • Diets low in salt and medication to treat swelling of the stomach and feet caused by fluid retention 
  • Vitamin supplements since people with primary sclerosing cholangitis often do not have enough vitamins A, D and K 
  • Medications (such as Cholestyramine and Ursodiol) to control itching caused by too much bile in the bloodstream, and to improve bile flow

Endoscopic or surgical procedures may be used to open major blockages in bile ducts. A catheter is a thin, flexible tube used to drain bile from the ducts and relieve the obstruction. (See ERCP above.)

A prosthesis (artificial device in the form of a hollow tube) may be placed in the bile ducts after they have been opened in order to keep the ducts open. Some patients may undergo surgery in order to explore the bile ducts and open major blockages.

Liver transplantation may be discussed as a treatment option if the liver begins to fail. The child would be evaluated to see if they are a candidate for liver transplantation.

If a transplant is the best treatment option, the care team will focus on preventing complications and treating symptoms while awaiting an organ.

Sclerosing cholangitis is a disease that continues to advance and tends to become more severe over time.

Medication does not have a major impact on slowing the progression of primary sclerosing cholangitis, but it has an important role in treating complications. For instance, Ursodiol can help stop itching, but it does not help a patient survive longer, and it does not delay the time to referral for liver transplant.

Children with primary sclerosing cholangitis account for about 2 percent of all liver transplants done in children. Liver transplantation is successful in 90 percent of these patients, who go on to have a good quality of life after recovery.

Last Updated 08/2015