Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic liver disease caused by progressive inflammation and scarring of the bile ducts of the liver. The inflammation impedes the flow of bile to the gut, which can ultimately lead to liver cirrhosis, liver failure and liver cancer. Autoimmunity is believed to be the underlying cause of the inflammation.

Approximately three out of every 100,000 individuals in the world are diagnosed with sclerosing cholangitis. Males are twice as likely than females to get PSC. The disease normally starts from age 20 to 30, though may begin in childhood. PSC progresses slowly, so the disease can be active for a long time before it is noticed or diagnosed.

In the early stages of primary sclerosing cholangitis (PSC), you may not experience any symptoms at all. As the disease progresses, symptoms may come and go and are caused by the bile not being drained properly. This can affect liver function and cause the bile to seep into your bloodstream.

Symptoms may include:

  • Itching
  • Chronic fatigue
  • Jaundice, yellowing of the skin and eyes
  • Loss of appetite
  • Weight loss
  • Chronic fatigue
  • Chills
  • Fever
  • Upper abdominal tenderness

Bile assists in the breakdown and absorption of fat. The absence of bile leads to fat malabsorption and deficiencies of fat-soluble vitamins (A, D, E, K).

Sclerosing cholangitis is a disease that continues to advance and tends to become more severe over time. Medication does not have a major impact on slowing the progression of primary sclerosing cholangitis, but it has an important role in treating complications.

Children with primary sclerosing cholangitis account for about 2 percent of all liver transplants done in children. Liver transplantation is successful in 90 percent of these patients, who go on to have a good quality of life after recovery.

Currently there is no cure for primary sclerosing cholangitis. Treatment is directed at managing symptoms and opening narrowed bile ducts. Symptoms of this disease can sometimes be managed by:

  • Antibiotics to treat bile ducts that have become infected
  • Diets low in salt and medication to treat swelling of the stomach and feet caused by fluid retention
  • Vitamin supplements since people with primary sclerosing cholangitis often do not have enough vitamins A, D and K
  • Medications (such as Cholestyramine and Ursodiol) to control itching caused by too much bile in the bloodstream, and to improve bile flow

Endoscopic or surgical procedures may be used to open major blockages in bile ducts. A catheter is a thin, flexible tube used to drain bile from the ducts and relieve the obstruction. A prosthesis (artificial device in the form of a hollow tube) may be placed in the bile ducts after they have been opened in order to keep the ducts open.

Liver transplantation may be an option if the liver begins to fail. Liver transplantation is very effective in the treatment of patients with advanced liver disease caused by primary sclerosing cholangitis. If a transplant is the best treatment option, the care team will focus on preventing complications and treating symptoms while awaiting a donated liver.