Primary Sclerosing Cholangitis (PSC)

Primary sclerosing cholangitis (PSC) is a rare disease that attacks the bile ducts. The word sclerosing means scarring. In PSC, your bile ducts become scarred and gradually narrow until bile backs up into your liver and starts to damage it.

Bile is an important digestive juice that you need to breakdown fats. It is also needed to absorb the fat-soluble vitamins A, D, E, and K. Bile, which is made in your liver, travels through ducts from your liver to your gallbladder and small intestine. PSC damages the bile ducts both inside and outside your liver.

Nobody knows the exact cause of PSC. It can start at any age. Healthcare providers usually diagnose it at about age 30 to 40. PSC is more common in men.

Some of the possible causes include:

• Genes. Having a family member with PSC increases your risk.
• Germs. Bacteria and viruses may play a role in triggering PSC.
• Inflammatory bowel disease. Many people with PSC also have ulcerative colitis — an inflammatory bowel disease.
• Immune system problems. Your immune system is your body's defense system. An abnormal immune system may play a role in PSC.

Symptoms of PSC develop slowly. You may have PSC for many years without any symptoms. Once symptoms develop, they may come and go, but they usually get worse over time. Bile that backs up into your liver may eventually cause a type of liver scarring known as cirrhosis, which leads to liver failure. Symptoms of PSC include:

• Itchy skin
• Fatigue
• Abdominal pain
• Yellowing of the skin and eyes, called jaundice
• Chills and fever from infection of your bile ducts

Your healthcare provider may suspect PSC if a routine blood test shows abnormal liver enzymes. These enzymes increase when there is liver damage. An imaging study of the bile ducts will likely confirm the diagnosis. Tests may include:

• Endoscopic retrograde cholangiopancreatography. In this procedure, your healthcare provider injects dye into your bile ducts, via an endoscope. This is a long, thin telescope he or she inserts through your mouth, and down into your small intestine. Your healthcare provider takes X-ray pictures after the dye is injected. He or she may open blocked bile ducts during this procedure.
• Percutaneous transhepatic cholangiography. In this study, your healthcare provider injects dye into your bile ducts, via a long needle he or she inserts through your skin. Your healthcare provider takes X-rays after injecting the dye.
• Magnetic resonance cholangiopancreatography. This study uses a computer and radio waves to make images of your bile ducts. If your healthcare provider uses dye, he or she injects it into your blood vessel. It is the main test used to diagnose PSC.
• External or internal ultrasound (an endoscopic ultrasound, EUS). These studies use ultrasound waves to make pictures of the bile ducts and drainage system as well as the gallbladder and liver.
• Liver biopsy. This test takes a sample of the liver tissue for evaluation under a microscope. It is sometimes necessary to evaluate for other similar diseases but is usually not needed.
• Blood tests. These can help diagnose a bile duct disorder. They can also evaluate for vitamin deficiency.

There is no known treatment to stop or slow down PSC. Your healthcare provider can treat symptoms with medicines to relieve itching and treat infection. He or she can also sometimes open the ducts in your liver if they become blocked. You may need to take vitamin supplements as well. PSC progresses slowly, but after about 10 to 15 years, it may eventually lead to liver failure. Many people with liver failure respond well to liver transplant surgery.

Osteoporosis is also very common with PSC. You will need to have your bone density monitored. Vitamin levels will also need to be monitored. If you have inflammatory bowel disease (IBD), you should have a colonoscopy every year. Even if you do not have IBD, a colonoscopy should be done when you are diagnosed with PSC to evaluate for colitis. People with PSC are also at risk for cancers of the bile duct, liver, and colon. Your doctor can discuss screening tests with you.

Learn as much as you can about your disease and work with your medical team to manage the condition. Avoid alcohol and maintain a healthy diet. Tell your healthcare provider about any medicines you are taking, including over-the-counter medicines and any herbal or dietary supplements.

• Primary sclerosing cholangitis is a rare disease that attacks your bile ducts.
• Symptoms of PSC develop slowly and might include itchy skin, fatigue, abdominal pain, yellow skin, or chills and fever if an infection exists.
• There is no known cure. But, medicine can ease symptoms.
• If liver failure occurs, you may be a candidate for a liver transplant.

Tips to help you get the most from a visit to your healthcare provider:

• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells you.
• At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your provider if you have questions.