Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic disease that slowly causes long-term damage to the bile ducts.

your quick guide to this page

what is primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is a liver disease in which the bile ducts (small tubes through which bile flows from the liver to the gallbladder and intestines) progressively decrease in size due to inflammation and scarring.  This causes the bile that is normally carried out of the liver to accumulate within the liver, which can lead to liver damage. This disease may only affect the bile ducts within the liver but often times can affect the large bile ducts outside the liver as well.

fast facts

  • Primary sclerosing cholangitis affects 1 in every 100, 000 people.
  • Men are more likely to have primary sclerosing cholangitis than women.
  • The average age of diagnosis for patients with primary sclerosing cholangitis is 40 years old.
  • 80% of people with primary sclerosing cholangitis also have inflammatory bowel syndrome.
  • People with primary sclerosing cholangitis have a 10-20% chance of developing bile duct cancer.

symptoms

Primary sclerosing cholangitis is unpredictable and not well understood. A person can have the disease for many years before symptoms develop. When a patient does experience symptoms they may include:

  • Itching
  • Fatigue
  • Jaundice (yellowing of the skin and eyes)
  • Episodes of fever, chills and abdominal pain which may indicate an infection within the bile duct.

If a person who suffers from primary sclerosing cholangitis develops fever and chills, they should go to the hospital immediately.  Eventually, in the advanced stages of PSC, cirrhosis may develop with signs of liver failure.

lifestyle impacts

The impact of primary sclerosing cholangitis on a patient can be unpredictable. Many people are able to live a normal life with PSC while others progress to advanced liver damage and eventually liver failure. If symptoms begin to develop, common symptoms like fatigue, pain, and severe itching can make it difficult for people with this condition to carry out daily tasks.

what can I do?

prevention

The cause of PSC is still unknown along with ways to prevent it. Some research suggests it may be a result of bacterial or viral infections. Genetic factors may also play a role in developing this disease.

diagnosis

Since many patients with PSC do not experience any symptoms, diagnosis is often found through routine blood tests. For patients that are experiencing symptoms, a cholangiography X-ray test or MRI are used to diagnose a patient with PSC.

treatment

There currently is no known specific treatment for primary sclerosing cholangitis.  However, symptoms of this disease can be treated with varying success by antibiotics, vitamins, and medications to control itching and improve bile flow. When the disease has progressed to liver failure, liver transplantation may be considered.

additional resources

Here are a few questions to ask your doctor or medical team:

  • What is the status of my liver?
  • What treatments do you recommend to slow the progression of the disease?
  • How can I relieve the symptoms I am experiencing?
  • Will I need a liver transplant?