Primary Sclerosing Cholangitis (PSC)

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Overview
Symptoms and Diagnosis
Treatment

What Is Primary Sclerosing Cholangitis (PSC)?

PSC is a chronic disease in which your bile ducts and liver get inflamed and swollen.

Over time, the bile ducts harden and become blocked causing bile to build up in the liver. This causes liver damage and can even lead to liver failure.

Damage can occur quickly or over many years, and doctors can't reverse it. Your doctor will focus on slowing down liver damage for as long as possible.

PSC is a relatively rare disease.

About 1 in 10,000 people have PSC. Doctors diagnose this disease in about 1 in 100,000 people per year worldwide.

Primary sclerosing cholangitis causes

Doctors don't know exactly what causes PSC.

The disease likely comes from an unknown combination of genetics and environment. This means your genes may make you at risk of getting the disease.

PSC risk factors and complications

PSC affects at least twice as many men as women. Most people with this disease receive their diagnosis sometime between the ages of 30 and 50.

About 3 out of 4 people with PSC also have inflammatory bowel disease. This is most often ulcerative colitis, a severe inflammation of the intestines that causes ulcers and stomach pain.

People with PSC also:

Are more likely to have an autoimmune disorder like type 1 diabetes. But the reason for this connection remains unclear.
Have a greater risk for bile duct cancer.
May develop osteoporosis, a thinning of the bones that leaves them prone to fractures. That's because, without adequate bile, the body can't digest fat-soluble vitamin D, which helps protect bones.

Left untreated, PSC can lead to liver failure.

How to prevent primary sclerosing cholangitis

Doctors can't currently prevent someone from getting PSC.

This might change in the future if they can decode the genetics of this disease.

Why choose UPMC's Center for Liver Care for primary sclerosing cholangitis care?

PSC is a serious chronic condition for which there is no cure. That means you will be dealing with the effects of this disease for decades.

UPMC's liver disease experts can help you slow the progression of your disease.

If your PSC leads to liver failure, you will need a liver transplant. Our experts partner closely with UPMC's world-renowned liver transplant. UPMC is 1 of the oldest and most skilled transplant centers in the country.

Also, UPMC is a national leader in living-donor liver transplants. By identifying a living donor, you may be able to receive a transplant sooner.

Primary Sclerosing Cholangitis (PSC) Symptoms and Diagnosis

Even though PSC is a serious disease. Many people who have it don't have symptoms right away.

As the disease worsens, symptoms can come and go.

PSC symptoms

Symptoms of PSC include:

Pain in the upper right part of the belly.
Extreme itching.
Unexplained weight loss.
Yellowed skin or eyes known as jaundice.
Fatigue.
Chills.
Night sweats.

See your doctor if you have these symptoms. They could have many causes, so expect your doctor to run a range of tests to confirm a diagnosis.

Diagnosing primary sclerosing cholangitis

Doctors may suspect PSC when routine blood tests have abnormal results, even if you don't have symptoms.

Your doctor will order tests to help diagnose PSC, including:

Blood tests.
MRI scans.
Endoscopy — using a scope to see inside a person's digestive system.
Endoscopic ultrasound and endoscopic retrograde cholangiopancreatography (ERCP). This combines endoscopy with dye and X-ray technology to view the bile ducts.
FibroScan® — a noninvasive liver ultrasound to determine how much fibrosis your liver has.
Liver biopsy — surgery to remove a small piece of liver to look at it under a microscope.

Primary Sclerosing Cholangitis (PSC) Treatment

If you have PSC, your doctor will:

Track your liver health closely to find and treat any new problem right away.
Treat any infections and manage symptoms like pain or jaundice.

Lifestyle changes to treat PSC

Lifestyle changes to help manage PSC include:

Avoiding alcohol.
Quitting smoking.
Maintaining a healthy weight.
Eating a variety of fruits, veggies, and whole grains.
Getting vaccines for hepatitis A and B.

Medicine to treat primary sclerosing cholangitis

People with PSC are prone to getting liver-related infections. Your doctor will prescribe antibiotics in this case.

Your doctor will also help you manage pain if needed.

Surgery to treat PSC

If your bile ducts become blocked, your doctor may need to do minor surgery.

Types of surgery to open the ducts so that bile will no longer build up in your liver include:

Endoscopy: using a scope to open the bile ducts.
Esophageal variceal banding: wrapping a small band around blood vessels in the liver to prevent internal bleeding.
Paracentesis: withdrawing extra fluid through the abdominal wall using a hollow needle.
Thoracentesis: using a needle to withdraw fluid from the chest or lungs.
Transjugular intrahepatic portosystemic shunt (TIPS): inserting a small coil or stent into a vein in the liver. TIPS reduces liver hypertension or the risk of bleeding.

If PSC causes severe cirrhosis or liver failure, you may need a liver transplant. Transplant provides the only known cure for PSC.

We work closely with the experts at UPMC Liver Transplant Services and guide you through each of the liver transplant process.