Primary Sclerosing Cholangitis (PSC) is a chronic disease that slowly damages the bile ducts in the liver. Bile is a liquid that helps with digestion, and it travels through the bile ducts to the gallbladder and small intestine, where it helps break down fats and fatty vitamins.
In PSC patients, the bile ducts get blocked due to inflammation and scarring. This causes the bile to build up in the liver, leading to damage to liver cells and the development of cirrhosis or fibrosis in the liver. As cirrhosis progresses and more scar tissue forms, the liver gradually loses its ability to function properly. The build-up of scar tissue can also block the drainage of bile, leading to bile infections.
PSC is a slow-progressing disease, and many patients may have it for years before experiencing any symptoms. The symptoms can remain stable, come and go, or progress gradually. It may take 10-15 years or even longer for liver failure to occur after diagnosis. Eventually, many people with PSC will require a liver transplant, typically about 10 years after being diagnosed. PSC can also increase the risk of bile duct cancer. Endoscopy and MRI tests may be used to monitor the progression of the disease.
Quick Facts
Information for Newly Diagnosed Individuals: What are the symptoms of PSC? Many people with PSC do not experience symptoms, especially in the early stages. When symptoms do occur, the most common ones are fatigue, itching of the skin (pruritus), and yellowing of the skin and eyes (jaundice). These symptoms may come and go, but they can worsen over time. Bile duct infections can also occur, leading to episodes of fever, chills, and abdominal pain.
What causes PSC?
- The exact cause of PSC is unknown. It is more common in men, and it may be related to bacterial or viral infections and immune system problems. Genetic factors may also play a role. Recent studies suggest that PSC may be more common than previously thought.
How is PSC diagnosed?
- Since many PSC patients don’t experience symptoms, the disease is often discovered through abnormal results on routine liver blood tests. A formal diagnosis is usually made through cholangiography, which is an X-ray test where dye is injected into the bile ducts, or through MRI scans.
How is PSC treated?
- There is no cure or specific treatment for PSC. Medication can help relieve the itching associated with the disease, and antibiotics are used to treat bile duct infections. Most people with PSC need to take vitamin supplements. In some cases, bile duct surgery or endoscopy may be performed to temporarily improve bile flow.
When is a liver transplant necessary?
- Over time, many PSC patients will experience a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be required. The survival rate after transplantation is excellent, with about 80% of patients surviving for two or more years and having a good quality of life.
Who is at risk for PSC?
- PSC is slightly more common in men and usually affects people between the ages of 30 and 60. As many as 75% of PSC patients also have inflammatory bowel diseases, particularly ulcerative colitis.
What is being done to find a cure for PSC?
- PSC has been known for 100 years, but now doctors can diagnose it early, allowing treatment to start before severe liver damage occurs. Scientists continue to study the disease to find its cause and understand its development. Drug therapy trials involving many patients worldwide are also exploring the use of medications to relieve symptoms and control liver damage.
Questions to Ask Your Doctor:
- How severe is the liver damage?
- What treatments do you recommend, and will they slow down the progression of the disease?
- Will I need medication, and what are the potential side effects?
- Should I make any changes to my diet?
- Are there any supplements you recommend?
- How can I find relief from my symptoms?
- Is screening for bile duct cancer necessary at this time?