What is Primary Sclerosing Cholangitis ?
Primary sclerosing cholangitis (PSC) is a rare liver disease that damages the bile ducts inside and outside the liver. Inflammation occurs in the bile ducts causing them to become scarred and narrowed until they eventually become blocked. These blockages prevent the flow of bile which is needed for digestion. As bile becomes trapped in the liver, the liver becomes damaged. Over time this can cause fibrosis and cirrhosis of the liver and eventually liver failure.
PSC progression differs for each person, but it helps to know the basic facts. PSC Partners Canada provides resources and support to help you understand the disease.
Find out more about PSC
How is PSC diagnosed ?
PSC can be diagnosed at various stages of progression, depending on how a person with PSC experiences symptoms or if a person is being followed closely by a doctor for other reasons. Many people go undiagnosed for years and only find out there is something wrong through a routine liver blood test. Some are diagnosed because they are having PSC-related symptoms, even if their PSC may still be at an early stage. And some will be diagnosed because they have advanced PSC, and symptoms of advanced PSC have started.
When PSC is suspected, a medical provider follows a process to confirm the diagnosis. Here’s how it typically works:
- Initial check-up: The doctor starts by reviewing the patient’s medical history, asking about symptoms, and doing a physical exam. They also check blood tests to see if the liver is working properly.
- Imaging test (MRCP): A special type of MRI scan called an MRCP is done to give a detailed picture of the bile ducts. If the scan shows narrowing or blockages (called “strictures”) in the bile ducts — and doctors rule out other possible causes — this is usually enough to diagnose PSC.
- Unclear scan results: If the scan isn’t clear, they might do another scan in about a year or take a small sample of liver tissue (called a biopsy) to get more information.
- Normal scan but ongoing symptoms: If the MRCP looks normal but the patient still has signs of liver disease, a liver biopsy may be done. This helps doctors check for “small-duct PSC” (a less common form of the disease) or see if something else might be causing the symptoms.
What should I expect when diagnosed with PSC ?
Everyone’s PSC journey is different. In general, PSC is a chronic progressive disease that currently has no treatment that has been proven effective at slowing PSC progression or providing a cure. There are however treatments and medical interventions that may help with symptoms or complications related to PSC.
It is not easy to predict the rate of progression for any one person. Some patients need transplants sooner, and some never need a transplant.Typically, PSC progresses slowly, with current studies estimating the average time from diagnosis to liver transplantation to be 15-20 years.
You may be experiencing symptoms such as abdominal pain, itching, or fatigue or you may not have any symptoms at all. It is important to familiarize yourself with the disease to recognize the signs of PSC. One complication that is important to know about is acute cholangitis, a bacterial infection of the bile ducts, that can develop at any stage of PSC and requires prompt treatment.
Find out more about PSC symptoms. Find out more about PSC complications.
Find a support community when you are ready
If you are newly diagnosed with PSC, we can help.
- PSC Partners offers two support groups on Facebook – open and closed
- The Resources section of this website provides many resources to help answer your questions.
- Patient stories gives a first-person perspective on living with PSC.
- Those living with PSC or their caregivers can request a community mentor
- Join peer-to-peer virtual ZoomRoom support gatherings or attend an event to network with other people diagnosed with PSC
Key facts about PSC
- PSC does not discriminate based on age, race, or gender.
- PSC is considered a rare disease in Canada, impacting over 4,000 Canadians in 2024.
- More than 75% of PSC patients have inflammatory bowel disease (IBD) , either ulcerative colitis (UC) or Crohn’s disease.
- PSC also is associated with other autoimmune diseases.
- PSC Partners Canada funds research that is aligned with our mission to drive research towards treatments and a cure and to provide support to those affected by PSC.
Related Resources
Some individuals affected by primary sclerosing cholangitis (PSC) may encounter few, if any, symptoms. Those who do experience symptoms may experience the following common symptoms:
PSC Alert Sheet Acute Cholangitis – Canadian Version Left untreated, acute cholangitis in PSC can cause serious complications, such as sepsis, jaundice and liver decompensation. This PSC Alert Sheet is...
