• By Liza Torborg

Mayo Clinic Q & A: Regular monitoring can help manage symptoms of PSC

March 7, 2015

DEAR MAYO CLINIC: My husband, 48, was diagnosed last month with primary sclerosing cholangitis. Does this mean he will eventually need a liver transplant, or do some people with the disease never get to that point? He has had irritable bowel syndrome for years; are the two conditions related?

Graphic Illustration of PSC

Primary Sclerosing Cholangitis

ANSWER: Primary sclerosing cholangitis, or PSC, is not associated with irritable bowel syndrome. But it can be related to another condition broadly termed inflammatory bowel disease, or more specifically, ulcerative colitis. Your husband should be tested for ulcerative colitis if that has not already been done. Many people who have PSC do eventually need a liver transplant. That is not the case for everyone, though. Regular monitoring and follow-up care can help manage symptoms and catch complications of PSC early.

PSC affects the ducts that carry the digestive liquid bile from your liver to your small intestine. In people who have PSC, inflammation causes scars within the bile ducts. The scars make the ducts hard and narrow. Over time, this can cause serious liver damage.

PSC often progresses slowly. As it advances, the disease may result in repeated infections and can lead to bile duct tumors or liver tumors. Eventually, PSC may cause the liver to fail. On average it takes about 10 years until most people with PSC need a liver transplant. However, the rate at which PSC progresses varies widely. Some people with this disease live a normal lifespan without every progressing to liver failure or needing a transplant.

To manage his care going forward, your husband should work with a hepatologist: a physician who specializes in liver disorders. Based on a number of laboratory and clinical factors, that specialist can help your husband calculate his risk for liver failure. He or she can also coordinate the ongoing monitoring your husband needs.

If he has never had a colonoscopy, your husband should talk to his hepatologist about having one now in order to determine if he has underlying ulcerative colitis. Random biopsies of tissue in the colon are recommended even if the mucosa appears normal, since ulcerative colitis in people with PSC may be very mild at first and may not be apparent without tissue specimens.

In addition to monitoring his condition, a hepatologist can also help your husband manage symptoms and complications of PSC, such as fatigue, itching and infections. At this time, a liver transplant is the only treatment known to cure PSC. During a liver transplant, surgeons remove the diseased liver and replace it with a healthy liver from a donor. Liver transplant generally is reserved for people with liver failure or other severe PSC complications. Though uncommon, it is possible for PSC to return even after a liver transplant.

If possible, your husband should consider consulting with a hepatologist at an academic medical center that has a strong hepatology division. Specialists at these centers can offer him the most up-to-date care. In addition, these centers often have access to research trials that are focused on new treatment options.

Although there is currently no specific medical therapy approved for PSC, a number of research trials studying PSC treatment are underway and more are coming soon. These trials can be an excellent way for people who have PSC to get treatment they may benefit from years before those agents are approved by the Food and Drug Administration. Elizabeth Carey, M.D., Gastroenterology & Hepatology, Mayo Clinic, Scottsdale, Ariz.