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Mayo Clinic physicians were among the first to describe the condition of primary sclerosing cholangitis (PSC). Mayo Clinic physicians treat more than 900 adults and children with this condition each year and have made many contributions to research about PSC. The team of specialists who treat patients includes hepatologists (liver specialists who diagnose diseases of the liver and oversee care and treatment), radiologists, pathologists, oncologists, pediatric specialists and surgeons.
Bile duct cancer is a major risk for individuals with PSC. Mayo is one of the few medical centers in the country with a protocol for treating bile duct cancer with a liver transplant and is a national referral center for PSC transplants. Mayo is world-renowned for its transplant programs.
Most often, PSC is first suspected after an abnormality shows up in a routine blood test or health evaluation. Mayo specialists use blood tests, cholangiography and liver biopsies to diagnose PSC. They also look for symptoms such as jaundice, diarrhea, fatigue, itching and episodes of fever and chills. Once the diagnosis is made, physicians determine the stage of the disease. Read more about primary sclerosing cholangitis diagnosis.
There is no cure for PSC; treatment focuses on controlling symptoms and slowing its progression. Medications may be prescribed for itching or infection, vitamin supplements for nutritional deficiencies. Endoscopic procedures are sometimes used to open blockages and improve bile flow. If the disease progresses to severe scarring of the liver, causing liver failure, patients may need a liver transplant. Mayo Clinic has research trials evaluating the treatment of PSC. Read more about primary sclerosing cholangitis treatment options.
Primary sclerosing cholangitis is a disease in which the ducts inside and outside the liver are narrowed due to inflammation. In the disease's early stages, the bile ducts (bile is a liquid that helps break down fat in food) and the tissues around them become inflamed. With the spread of inflammation, the bile ducts are destroyed and scar tissue is created. Continued inflammation and scar tissue buildup can lead to cirrhosis, where scar tissue distorts the liver, disrupts blood flow through the liver and interferes with its normal function.
PSC most often affects people in their 30s to 50s; the average age at diagnosis is 40. However, it can arise in childhood. About 60 to 75 percent of people diagnosed with the disease are men. Approximately 70 percent of people with PSC have an associated disease such as inflammatory bowel disease (IBD), osteoporosis, gallbladder disease and bile duct cancer (or cholangiocarcinoma). It is worth noting, though, that only 1 to 5 percent of people with inflammatory bowel disease have PSC.
The cause of PSC is unknown, but it is thought to be an immune response in which the body attacks the cells lining the bile ducts of the liver. This response may be due to exposure to something in the environment, a virus or bacteria, or a person's genetic makeup.
The course of PSC is unpredictable, but in most people it progresses slowly. Although PSC can be life-threatening, many people with the disease live active, productive lives.
John wants others who receive diagnoses of bile duct cancer to know there is hope.
Read John's story.
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