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.

Primary sclerosing cholangitis damages the hepatic, cystic, and common bile ducts, which carry bile out of the liver.

In primary sclerosing cholangitis, 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.

Causes of Primary Sclerosing Cholangitis

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

Primary Sclerosing Cholangitis is linked to inflammatory bowel disease (IBD). About three out of four people with Primary Sclerosing Cholangitis have a type of inflammatory bowel disease called ulcerative colitis. The link between Primary Sclerosing Cholangitis and inflammatory bowel disease is not yet understood.

People Who Get Primary Sclerosing Cholangitis

Most people with Primary Sclerosing Cholangitis (PSC) are adults but the disease also occurs in children. The average age at diagnosis is 40 years old. PSC is more common in men than women. Having family members with PSC may increase a person’s risk for developing PSC.

Symptoms of Primary Sclerosing Cholangitis

The main symptoms of Primary Sclerosing Cholangitis (PSC) are:

• 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.

Complications of Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis (PSC) can lead to various complications, including:

• Deficiencies of vitamins A, D, E, and K
• Infections of the bile ducts
• Cirrhosis – extensive scarring of the liver
• Liver failure
• Bile duct cancer

Diagnosis of Primary Sclerosing Cholangitis

Blood tests to check levels of liver enzymes are the first step in diagnosing Primary Sclerosing Cholangitis (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)
  Endoscopic Retrograde Cholangiopancreatography uses an endoscope – a long, flexible, lighted tube – that goes down the mouth, beyond the stomach, and into the duodenum (pronounced – doo-AW-duh-num – is the first part of the small intestine, right after your stomach) 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. Endoscopic Retrograde Cholangiopancreatography 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)
  Magnetic Resonance Cholangiopancreatography 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. Magnetic Resonance Cholangiopancreatography 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.

Treatment For Primary Sclerosing Cholangitis

Although researchers have studied many treatments, none has been shown to cure or slow the progress of Primary Sclerosing Cholangitis (PSC). Treatment of Primary Sclerosing Cholangitis aims to relieve symptoms and manage complications. Medical treatment may include:

• Various medications to relieve itching
• Antibiotics to treat infections
• Vitamin supplements

Instruments passed through an endoscope during Endoscopic Retrograde Cholangiopancreatography (ERCP) can help open blocked bile ducts.

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

For More Information About Primary Sclerosing Cholangitis

American Liver Foundation
75 Maiden Lane, Suite 603
New York, NY 10038-4810
Phone: 1-800-GO-LIVER (465-4837) or 212-668-1000
Fax: 212-483-8179
Email: info@liverfoundation.org
Internet: www.liverfoundation.org

National Digestive Diseases Information Clearinghouse (NDDIC)
2 Information Way
Bethesda, MD 20892-3570
Phone: 1-800-891-5389
TTY: 1-866-569-1162
Fax: 703-738-4929
Email: nddic@info.niddk.nih.gov
Internet: www.digestive.niddk.nih.gov

Source: digestive.niddk.nih.gov – June 2008