PSC FAQs

What is PSC?

Primary sclerosing cholangitis, or PSC for short, is a rare, autoimmune, non-alcohol-related, progressive liver disease that attacks the bile ducts in and outside the liver. 

Your bile ducts transport bile (a fluid that helps digest food) from the liver to the intestines. When the ducts are inflamed, they can’t work properly; this leads to scarring and narrowing of the ducts, which can eventually lead to blockages.

Blockages trap bile inside the liver, which further progress the disease toward fibrosis (early stages of scar tissue) and cirrhosis (severe scar tissue and impaired liver functions). This leads to liver failure and the need for transplant. Currently there is no cure, though there is hope for a treatment with vancomycin.

What Are the Symptoms of PSC?

• Pain near the liver behind the ribs
• Elevated liver numbers
• Intense itching (pruritus) anywhere on the body. (This is caused by bile acids entering the bloodstream. It is technically your blood that is itchy).
• Vomiting
• Fever
• Chills and night sweats
• Cholangitis attacks (bacterial infection in the bile ducts)
• Jaundice (yellowing of the skin)

Some people have no symptoms at all, particularly in the early stages.

Additionally, if you have IBD, a sign that you might have PSC is when you lose response to your IBD meds, both oral and biologic. Some patients lose response to their biologic without developing antibodies.

What Are the Stages of PSC?

PSC is a progressive disease. The rate it progresses affects everyone differently. 

However, PSC can be divided into four stages. Each stage lasts about five years on average, however this can vary drastically from person to person.

1. Stage 1: Cholangitis or portal hepatitis: degeneration of bile ducts and inflammation
2. Stage 2: Periportal fibrosis or periportal hepatitis: fibrosis (scar tissue) continues but they’re usually in separate part so the liver
3. Stage 3: Septal fibrosis, bridging necrosis or both: fibrosis is more widespread, and begin to connect with each other
4. Stage 4: Biliary cirrhosis: Widespread, honeycomb like scarring known as cirrhosis in which parts of the liver no longer function

Who is at Risk for Developing PSC?

Only about 30,000 people in the US have PSC, so it is officially deemed a rare disease. 

PSC is most commonly found in people who live with inflammatory bowel disease (IBD). About 80% of people with PSC have already been diagnosed with IBD (much more commonly ulcerative colitis (80%) than Crohn’s disease (10%) or indeterminate colitis (10%)). However, only about 5% of people with IBD will develop PSC.

Statistically, PSC affects more men than women, and people are typically diagnosed between the ages of 30 – 50, but children and adolescents can also be affected. Those who have Northern European heritage are also at higher risk.

Why is PSC Linked to IBD?

Even though 80% of PSC cases are found in individuals with IBD, doctors and researchers don’t know why or how the two autoimmune diseases are linked. Theories suggest that the gut microbiome may play a large part in both diseases, but more research needs to be done. Other speculations suggest that individuals with IBD and PSC have a certain genetic profile, and the diseases are exacerbated by things like environmental triggers or bacteria.

What Are the Risks of PSC?

PSC increases the risk of colorectal cancer and gallbladder cancer, as well as cancer of the bile ducts, called cholangiocarcinoma, which affects 5-10% of PSC patients.

How is PSC diagnosed and monitored?

Elevated liver function tests can be a sign that someone has PSC. Common liver enzymes that may indicate you have PSC include:

• Bilirubin
• GGT
• AST
• ALT
• Alkaline phosphatase

Other tests are often needed to confirm an official diagnosis. These tests can include:

• Magnetic Resonance Cholangiopancreatography (MRCP): A special type of MRI, which takes images of your intestines using magnetic waves
• Endoscopic Retrograde Cholangiopancreatography (ERCP): An invasive procedure that involves sending a scope into your small intestine
• Abdominal Ultrasound: Ultrasounds can monitor the liver and gallbladder for signs of cirrhosis or focal masses. It also provides a measure of the diameter of the common bile duct and sometimes can detect dilatation of intrahepatic ducts.
• FibroScan: A special type of ultrasound that measures the elasticity of your liver to identify the level of fibrosis. 
• Liver Biopsy: Under local anesthetic, a physician will remove tissue samples from your liver. Some are completed by inserting a needle between the ribs, while another type involves sending a needle down through the transjugular vein.

Since everyone’s PSC journey is so unique, work with your medical team to determine the types of testing and frequency to monitor your condition. Many patients get regular blood work to watch their liver enzymes. Other patients get regular or as-needed MRCPs, ultrasounds, colonoscopies, and liver biopsies.

What are the current treatments?

There are currently no effective, FDA-approved drugs for PSC; however, the off-label drug vancomycin has been observed to slow and sometimes even stop the progression of the disease. Many patients on vancomycin experience elevated liver numbers return to normal, alleviation of their symptoms, and the stopping or reversal of their liver fibrosis!

In most patients, vancomycin has also eliminated the need for liver transplant; also, cholangiocarcinoma has not been seen in PSC patients who are taking vancomycin.

Vancomycin has several factors that affect its efficacy, so if you’re considering taking vancomycin, first read the best practices.

What about Ursodeoxycholic Acid?

Urso is also used off-label for PSC patients. It’s typically used to treat gallbladder disease and primary biliary cholangitis (PBC). Unfortunately, though Urso can return elevated liver numbers to normal, it is not actually proven to stop the progression of the disease.

What about Liver Transplants?

Without vancomycin, many PSC patients will require a liver transplant. While a transplant can certainly be life-saving and significantly increase the quality of life for some patients, PSC returns in about 20% of patients after transplant.

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