What Is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis (PSC) is a disease that affects your bile ducts. Bile is a digestive fluid your liver makes. The ducts let it flow from your liver to your gallbladder and finally to your small intestine. There it helps break down fat from food.

With PSC, your bile ducts become inflamed. This causes scars to form that block bile from traveling through them. As a result, it builds up inside your liver and damages your liver cells. It also causes scar tissue to form in your liver.

Over time, the scar tissue affects your liver's ability to work the way it should and can lead to cirrhosis and eventually liver failure.

PSC can happen at any age. But it's more common in adults than in kids and affects more men than women. About 70% of people with PSC are male.

Symptoms

PSC develops slowly. You may have it for years before you notice any symptoms. Some of the signs are:

As PSC worsens, your bile ducts may become infected. This can lead to fever, chills, and pain in your belly.

Causes

Doctors aren't sure what causes PSC. It may be a virus, bacteria, genes, or problems with your immune system.

It's linked to inflammatory bowel disease, primarily ulcerative colitis and, less often, Crohn's disease. About 75% of people with PSC have ulcerative colitis. Doctors don't know how or why these conditions are connected.

Diagnosis

Your doctor will want to take a sample of your blood to check the levels of your liver enzymes. Unusual levels of certain enzymes could be a sign that you have PSC.

To confirm it, you'll have an imaging test called cholangiography to give your doctor a closer look at your bile ducts. There are several different types of cholangiography:

  • Magnetic resonance cholangiopancreatography (MRCP).This uses powerful magnets and radio waves to make detailed images of your bile ducts. It's often a doctor's first choice because it's safe, painless, and non-invasive.
  • Endoscopic retrograde cholangiopancreatography (ERCP). Before the test, you'll receive a sedative so you stay calm and comfortable. Your doctor will put a long, flexible tube called an endoscope through your mouth and into your digestive tract. He'll put dye into your bile ducts and take an X-ray. The dye helps your bile ducts show up more clearly.
  • Percutaneous transhepatic cholangiography (PTC). Your doctor puts a needle through your skin, places a thin tube into a duct in your liver, and puts dye through it. She then takes an X-ray.

 

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Treatment

No medications have been shown to help with PTC, but researchers are working on this. You may want to ask your doctor if you qualify for any drug therapy trials. These test medicines that aren't yet approved to treat PSC, and they may help with your symptoms or help control liver damage.

If you have an endoscopic retrograde cholangiopancreatography, your doctor may use special instruments through the endoscope to open blocked bile ducts.

Otherwise, the goal is to help your symptoms and control complications that can occur. For example, your doctor may prescribe medications to ease itching, antibiotics to control an infection, or vitamin supplements to help with any deficiencies.

If you have liver failure, you may need a liver transplant. Most people with PSC who have one have very good outcomes and quality of life afterward.

WebMD Medical Reference Reviewed by Jaydeep Bhat, MD, MPH on March 18, 2019

Sources

SOURCES:

National Institute of Diabetes and Digestive and Kidney Diseases: "Primary Sclerosing Cholangitis."

American Liver Foundation: "Primary Sclerosing Cholangitis."

National Institute of Diabetes and Digestive and Kidney Diseases: "ERCP."

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