What is Primary Sclerosing Cholangitis (PSC)?

PSC is a rare, progressive disorder involving the large bile ducts of the liver. The walls of the bile ducts become inflamed over time which leads to narrowing from scarring. These areas of narrowed and scarred bile ducts are called strictures. PSC is most commonly diagnosed between the ages of 30 and 50 and is more common in men than in women.

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The resulting damage to the bile ducts leads to complications of advanced liver disease. Also, PSC is associated with severe infections of the bile ducts and liver called cholangitis. Lastly, there is increased risk for the development of certain types of cancers:

A majority of people with PSC also have inflammatory bowel disease (IBD). Therefore, PSC can be diagnosed in the context of symptoms related to IBD, such as diarrhea, bloody bowel movements and abdominal pain. Other symptoms of PSC may include yellowing of the skin and eyes (jaundice), severe itching (pruritus) and fevers and chills due to infections in the bile ducts.

What Causes PSC?

The exact cause of PSC is not known, but multiple factors are thought to be involved, including:

  • Autoimmune process
  • Inflammation due to chronic entry of bacteria into the liver and bile ducts
  • Poor and compromised blood flow (ischemia) to the bile ducts

Expert Diagnosis and Treatment for Primary Sclerosing Cholangitis

PSC can be diagnosed when the above symptoms or complications develop. Sometimes it is diagnosed based on abnormalities found with routine blood tests or imaging tests. A diagnosis of PSC will be confirmed with a specialized MRI of the liver, pancreas and bile ducts called a magnetic resonance cholangiopancreatography (MRCP). Sometimes, the diagnosis will be confirmed by a procedure called endoscopic retrograde cholangiopancreatography (ERCP), which can also treat areas of stricture in the bile ducts. It is important to make an appointment with a UH liver specialist right away to confirm or rule out PSC.

Due to the strong association between PSC and inflammatory bowel disease, colonoscopy is recommended at the time of PSC diagnosis to screen for IBD. Cancer screenings for colorectal cancer and cancer of the liver, bile ducts, and gall bladder are essential in the management of PSC due to increased risk. This will be a part of the care your liver specialist provides.

There is no known treatment to slow the progression of PSC; however, symptoms may be managed with medications to relieve the itching associated with the disease and antibiotics to treat bile duct infections when they occur. In some cases, patients may undergo procedures such to temporarily unblock or widen the affected bile ducts. For some patients, liver transplantation will be considered as a treatment option.

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