Primary sclerosing cholangitis (patient information)

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What is Primary sclerosing cholangitis?

PSC is a disease that damages and blocks bile ducts inside and outside the liver. Bile is a liquid made in the liver. Bile ducts are tubes that carry bile out of the liver to the gallbladder and small intestine. In the intestine, bile helps break down fat in food. In PSC, inflammation of the bile ducts leads to scar formation and narrowing of the ducts over time. As scarring increases, the ducts become blocked. As a result, bile builds up in the liver and damages liver cells. Eventually, scar tissue can spread throughout the liver, causing cirrhosis and liver failure.

What are the symptoms of Primary sclerosing cholangitis?

The main symptoms of PSC include

• itching
• fatigue
• yellowing of the skin or whites of the eyes

An infection in the bile ducts can cause chills and fever. PSC progresses slowly, so a person can have the disease for years before symptoms develop.

Other symptoms may include:

• Enlarged liver
• Enlarged spleen
• General discomfort, uneasiness, or ill feeling (malaise)
• Indigestion
• Loss of appetite

What are the causes of Primary sclerosing cholangitis?

The causes of PSC are not known. Genes, immune system problems, bacteria, and viruses may play roles in the development of the disease.

Who is at risk for Primary sclerosing cholangitis?

PSC is linked to inflammatory bowel disease (IBD). About three out of four people with PSC have a type of IBD called ulcerative colitis. Most people with PSC are adults but the disease also occurs in children. The average age at diagnosis is 40. PSC is more common in men than women. Having family members with PSC may increase a person’s risk for developing PSC.

How to know you have Primary sclerosing cholangitis (Diagnosis)?

Blood tests to check levels of liver enzymes are the first step in diagnosing PSC. Doctors confirm the diagnosis using cholangiography, which provides pictures of the bile ducts.

Cholangiography can be performed in the following ways:

• Endoscopic retrograde cholangiopancreatography (ERCP). ERCP uses an endoscope—a long, flexible, lighted tube—that goes down the mouth, beyond the stomach, and into the duodenum to reach an area in the digestive tract where dye can be injected into the bile ducts. X rays are taken when the dye is injected. ERCP also can be used to take a tissue sample or to treat blocked ducts.
• Percutaneous transhepatic cholangiography. This procedure involves inserting a needle through the skin and placing a thin tube into a duct in the liver. Dye is injected through the tube and x rays are taken.
• Magnetic resonance cholangiopancreatography (MRCP). MRCP uses magnetic resonance imaging (MRI) to obtain pictures of the bile ducts. MRI machines use radio waves and magnets to scan internal organs and tissues. MRCP does not involve using x rays or inserting instruments into the body. This safe and painless test is increasingly used for diagnosis.

Other testing may include ultrasound exams and a liver biopsy. Ultrasound uses sound waves to create images of organs inside the body. A biopsy involves removal of a small piece of tissue for examination with a microscope.

When to seek urgent medical care

Call your health care provider if you have jaundice and itching that do not go away.

Treatment options

Although researchers have studied many treatments, none has been shown to cure or slow the progress of PSC. Treatment of PSC aims to relieve symptoms and manage complications. Medical treatment may include various medications to relieve itching, antibiotics to treat infections, and vitamin supplements. Instruments passed through an endoscope during ERCP can help open blocked bile ducts.

Liver transplantation may be an option if the liver begins to fail.

Where to find medical care for Primary sclerosing cholangitis

Directions to Hospitals Treating Primary sclerosing cholangitis

What to expect (Outlook/Prognosis)

How well patients do varies. The disease tends to get worse over time and most patients develop:

• Biliary cirrhosis
• Liver failure
• Persistent jaundice

Some patients develop recurrent infections of the bile ducts. Some patients have worsening chronic liver disease with ascites and varices.

Patients with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma), and should be checked on a regular basis (usually each year) with liver scans and blood tests.

Possible Complications

• Cholangiocarcinoma
• Cholangitis (infection of the biliary system)
• Liver failure
• Vitamin deficiencies