About Primary Sclerosing Cholangitis (PSC)

By Kevin on

Name of the medical condition and other names that it’s been known by

  • Primary Sclerosing Cholangitis (PSC)

What is Primary Sclerosing Cholangitis (PSC)?

PSC is a disease that affects the bile ducts, which are tubes that carry bile from the liver to the small intestine. In PSC, these ducts become inflamed and scarred over time. This causes them to narrow and eventually become blocked. When this happens, bile can't flow properly and builds up in the liver, causing damage.

PSC is a chronic condition that lasts for a long time and worsens over time. It's believed to be an autoimmune disease, where the body's immune system attacks its own healthy cells by mistake. In this case, it attacks the bile ducts. The disease is more common in men and usually starts between ages 30 and 40. Many people with PSC also have inflammatory bowel disease, especially ulcerative colitis. PSC can lead to serious complications like liver failure, infections in the bile ducts, and an increased risk of certain cancers, especially bile duct cancer. There's no cure for PSC, but treatments can help manage symptoms and slow down liver damage. In severe cases, a liver transplant might be needed.

What are some symptoms?

Most people with PSC don't have symptoms at first. As the disease progresses, they might experience:

  • Itchy skin

  • Feeling very tired

  • Pain in the upper right part of the belly

  • Yellowing of the skin and eyes (jaundice)

  • Fever and chills

  • Unexplained weight loss

How is it diagnosed?

  • Blood tests: Doctors check for high levels of liver enzymes, which can show liver damage.

  • Imaging tests: Special pictures of the bile ducts are taken. The main test is MRCP (Magnetic Resonance Cholangiopancreatography), which uses strong magnets to create detailed images.

  • Liver biopsy: Sometimes, a small piece of liver tissue is removed and looked at under a microscope.

  • Endoscopy: A thin tube with a camera is used to look at the bile ducts from the inside. This is called ERCP (Endoscopic Retrograde Cholangiopancreatography).

  • Physical exam: The doctor checks for signs like yellowing of the skin or enlarged liver.

  • Medical history: The doctor asks about symptoms and other health conditions.

How is it treated?

  • Medications to relieve itching

  • Antibiotics to treat infections in the bile ducts

  • Vitamin supplements (A, D, E, and K) because PSC can make it hard for the body to absorb these vitamins

  • Procedures to open up narrowed bile ducts (done with endoscopy)

  • Liver transplant for severe cases

  • Regular check-ups to watch for complications like cancer

  • Lifestyle changes like not drinking alcohol and eating a healthy diet

Trend

  • In the United States, about 5 to 16 people out of every 100,000 have PSC.

  • There's no clear data on how many people have had it in the last five years.

  • We don't have a good five-year projection because PSC is rare and hard to predict.

History

Doctors first described PSC in the 1920s, but it wasn't well understood until the 1970s and 1980s. At first, doctors thought it was very rare. As they learned more about it and got better at diagnosing it, they realized it was more common than they thought. In the past, there weren't many treatments for PSC. Now, doctors can do more to help manage symptoms and slow down the disease. The biggest change came with liver transplants, which became an option for severe PSC in the 1980s. This has greatly improved the outlook for people with advanced PSC.

Source of Information

For informational purposes only. Consult a medical professional for advice.


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