What is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis (PSC) is a disease of the bile ducts in which the ducts become inflamed and harden or scar as result of the inflammation. The disease slowly progresses over time, and causes damage to the liver. The liver can eventually fail completely. The only known cure for primary sclerosing cholangitis is a liver transplant, while other treatment options focus on reducing the severity of symptoms.

There are often no associated symptoms in the early stages of primary sclerosing cholangitis. Abdominal pain, weight loss, itching, and jaundice can all be early warning signs of PSC. Physicians can usually detect abnormal liver function through a series of blood tests if PSC is suspected. X-rays or magnetic resonance imaging (MRI) may also be conducted on the bile ducts to determine if there is any damage. A sample of liver tissue can be taken via biopsy to assess the extent of any liver damage caused by PSC.

The exact cause of primary sclerosing cholangitis is not known. According to the Mayo Clinic, many doctors believe it may be caused by a reaction to infection or a toxin present in people predisposed to the disease. PSC occurs more frequently in men than in women. It is typically diagnosed in people between the ages of 25 and 45. Most people diagnosed with PSC also have some form of inflammatory bowel disease, such as ulcerative colitis or Chron's disease, though it is not known why the diseases occur together.

PSC patients almost always experience some level of cirrhosis of the liver, and many lose complete liver function as the condition progressively worsens. The time from diagnosis to liver transplant is typically 12 to 18 years. Frequent infections of the bile ducts are also a common complication associated with primary sclerosing cholangitis, due to the scarring of the bile ducts preventing bile from escaping the liver. PSC patients also have an increased risk of developing bile duct cancer and colon cancer.

Many PSC symptoms can be successfully treated and managed with a variety of medications and other procedures. Antihistamines and bile-acid-binding drugs are often prescribed to help control the itching many patients experience. Recurring infections are treated with various antibiotics, and vitamin supplements are often recommended due to the body's difficulty absorbing certain vitamins. Blocked bile ducts can be treated with surgical procedures involving the opening of the bile duct with a balloon catheter and the placement of plastic stents to keep the ducts open. A blockage may also be completely removed with the remaining ends of the duct being surgically reattached.