Primary sclerosing cholangitis (PSC) is caused by immune system-induced inflammation around the bile ducts within the liver, and the main bile duct that connects the liver to the small intestine. PSC develops slowly and affects slightly more men than women. It can also develop in adolescents.

The liver inflammation that develops in PSC causes scar tissue to form around the bile ducts. This leads to narrowing of the bile ducts, which may lead to infections and jaundice, and stops the flow of bile into the gut. The condition can eventually lead to liver cirrhosis or end-stage liver disease (liver failure). In addition, bile duct cancer may occur in some patients with this disease.

Many patients with PSC also suffer from inflammatory bowel disease (IBD). At Mount Sinai, patients with IBD have access to some of our most renowned gastroenterologists, who care for the greatest number of IBD patients in the country.

Research into IBD has been ongoing for at least 75 years. The condition was first identified at Mount Sinai, and significant discoveries have included a number of effective medications and a greater understanding of how the disease develops and progresses.

Because Mount Sinai is a major IBD research and treatment center, the Crohn's and Colitis Foundation has designated Mount Sinai a center of excellence for inflammatory bowel disease education for physicians. Mount Sinai liver disease researchers are currently examining the effectiveness of vitamin D to treat PSC. We are also exploring the relationship between PSC and exposure to environmental toxins, as well as genetic susceptibility to the disease.

Diagnosis

Diagnosis of PSC depends on laboratory blood work and a technique to help visualize the liver - either a noninvasive, magnetic resonance imaging technique known as MRCP (Magnetic Resonance Cholangiopancreatography) or, in some cases, an endoscopic procedure known as ERCP (Endoscopic Retrograde Cholangiopancreatography). A liver biopsy may also be necessary. Testing for fecal (stool) fat content is sometimes ordered when it appears that a nutritional disorder is also present due to the disease.

Treatment

The bile acid ursodeoxycholate (“Urso”) is often used to treat patients with PSC. However, this treatment is not successful for many patients.

Liver transplantation for PSC patients with severe liver impairment is a practical treatment option. While it is possible that the disease could recur after transplant, it would take a long time for severe damage to develop once again.

If patients with PSC develop bile duct cancer or liver cancer, they may be referred to Mount Sinai's Tisch Cancer Institute, a highly regarded surgical oncology center for the treatment of these diseases.