What Is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis (PSC) is a disease of the liver that causes inflammation and narrowing of the bile ducts inside and outside of liver. It's not certain what causes PSC, though it seems to be connected to other conditions. Some theories on the cause of PSC include bacterial and viral infection and the immune system's response. PSC is not thought to be directly inherited, but it is thought to have a genetic component.

Bile is necessary for the digestion of fat and to carry wastes out of the liver. PSC causes the bile ducts to narrow from scarring and inflammation, and bile begins to accumulate in the liver, which damages it. The scarring and the build-up of the bile prevent the liver from performing its important functions. Continued inflammation and scar tissue formation lead to cirrhosis of the liver, which is a condition that prevents the liver from filtering blood. Several years of PSC can lead to a cancerous tumor of the bile ducts called cholangiocarcinoma, which occurs in 10 to 15 percent of patients.

PSC does progress slowly in most cases, but it can also be unpredictable. People with PSC can receive treatment to relieve symptoms and help them to lead an active life, but the condition can be life threatening.

Who Gets PSC?

Most often, the people who are affected by PSC are between the ages of 30 and 60, with the average age of diagnosis being 40. PSC tends to be more common in men; 60 to 75 percent of people diagnosed are men. On the whole, PSC is an uncommon disease.

Symptoms of PSC

Many people discover that they have PSC through routine tests such as blood tests or regular check-ups. Some people have no symptoms at diagnosis or even for several years after.

Symptoms include:

• Diarrhea (which is caused from malabsorption of fat)
• Fatigue
• Fever/chills (from an infection in the bile ducts)
• Itching which often affects the entire body
• Jaundice (yellowing of the skin and eyes)

Diseases Associated with PSC

People with PSC are more likely to have inflammatory bowel disease (IBD) or osteoporosis. PSC more commonly occurs with ulcerative colitis, but also can be associated with Crohn's disease of the large intestine, sometimes called Crohn's colitis. The reason for the association with IBD is unknown, but it is thought to be the result of an immune response. As many as 70 percent of people with PSC also suffer from a related conditions.

Diagnosing PSC

PSC is typically diagnosed by a procedure called ERCP (endoscopic retrograde cholangio-pancreatography). During an ERCP, the physician will insert an endoscope into the mouth and navigate it through the body down to the ducts of the biliary tree. Dye is inserted into the ducts so that they will show up when X-rays are taken. The X-rays are then analyzed to determine if there are any problems with the bile ducts.

The diagnosis of PSC can also be somewhat confirmed by the a blood test for serum alkaline (ALK) phosphatase levels and serum aminotrasferases. The ALK test determines the amount of alkaline phosphatase that is in the blood, although there are many conditions that can alter normal ALK levels. Still, if the bile flow is being obstructed, alkaline phosphatase levels in the blood will be elevated.

A liver biopsy is also helpful to confirm the diagnosis of PSC, as well as to monitor the progress of the disease after diagnosis. A liver biopsy is done in a hospital setting on an outpatient basis with local anesthetic. The physician performing the test will use a needle to take a small sample of liver tissue for testing by a pathologist.

Treatment of PSC

There is no treatment that has been proven to be effective for treating PSC. Research to find an effective medical treatment is currently underway. Treatment plans focus on alleviating symptoms, interrupting the progression of the disease, and monitoring for potential complications.

The symptoms of PSC can be treated to make patients more comfortable. Itching may be treated with cholestyramine (Questran), diphenhydramine (Benadryl), or rifampin (Rifadin, Rimactane). For recurrent infections that may occur with PSC, antibiotics could be necessary. Because PSC interferes with that absorption of fat, supplements may be necessary to treat deficiencies of the fat-soluble vitamins A, D, and K. If blockages occur in the bile ducts, a surgical procedure may be necessary to stretch or open them. Stents, which keep the ducts open, may be placed in the ducts during this procedure.

If the progression of the PSC leads to liver failure, a liver transplant may be necessary. Liver transplants offer a good quality of life for recipients, as well as a survival rate of about 75 percent.

When to Call the Doctor?

If any of the following symptoms occur with PSC, call your doctor:

• Abdominal pain
• Black or very dark stools
• Jaundice
• Temperature over 100.4 degrees Fahrenheit or 38 degrees Celsius
• Vomiting with blood