What is primary sclerosing cholangitis?

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. They slowly narrow until bile backs up into your liver and starts to damage it.

Bile is an important digestive juice that you need to break down 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.

What causes PSC?

Nobody knows the exact cause of PSC. It can start at any age. Healthcare providers often 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.

What are the symptoms of 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 often get worse over time. Bile that backs up into your liver may over time cause a type of liver scarring known as cirrhosis. This leads to liver failure. Symptoms of PSC include:

• Itchy skin

• Extreme tiredness (fatigue)

• Belly pain

• Yellowing of the skin and eyes, called jaundice

• Chills and fever from infection of your bile ducts

How is PSC diagnosed?

Your healthcare provider may think you have 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 (ERCP). In this procedure, your provider injects dye into your bile ducts using an endoscope. This is a long, thin tube with a tiny camera and light on one end. The scope is put in through your mouth, and down into your small intestine. Your provider takes X-ray pictures after the dye is injected. They may open blocked bile ducts during this procedure.

• Percutaneous transhepatic cholangiography. In this study, your provider injects dye into your bile ducts using a long needle inserted through your skin. Your provider takes X-rays after injecting the dye.

• Magnetic resonance cholangiopancreatography (MRCP). This study uses a computer and radio waves to make images of your bile ducts. If your healthcare provider uses dye, they inject it into your blood vessel. This is the main test used to diagnose PSC.

• External or internal ultrasound (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 (biopsy) of the liver tissue. It's then checked under a microscope. This test is sometimes needed to evaluate for other similar diseases.

• Blood tests. These can help diagnose a bile duct disorder. They can also evaluate for vitamin deficiency.

How is PSC treated?

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. They 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 lead to liver failure. Many people with liver failure respond well to liver transplant surgery.

Brittle bones (osteoporosis) are also very common with PSC. You will need to have your bone density watched. Vitamin levels will also need to be watched. 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, gallbladder, and colon. Your provider can discuss screening tests with you.

Living with PSC

Learn as much as you can about your disease and work with your healthcare team to manage the condition. Don't drink alcohol. Be as physically active as you can and eat a healthy diet. Tell your healthcare provider about any medicines you are taking. This includes over-the-counter and prescription medicines, vitamins, herbs, and dietary supplements.

Key points about primary sclerosing cholangitis

• Primary sclerosing cholangitis is a rare disease that attacks your bile ducts.

• Symptoms develop slowly. They may include itchy skin, fatigue, belly pain, yellow skin, or chills and fever if an infection exists.

• Brittle bones (osteoporosis) are also very common with PSC. You will need to have your bone density watched.

• There is no known cure. But medicine can ease symptoms.

• A healthy lifestyle can help your physical and emotional well-being.

• If liver failure occurs, you may be a candidate for a liver transplant.

Next steps

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

• Know the reason for your visit and what you want to happen.

• 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 name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is advised and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.