Accessing the most up-to-date, accurate information is essential when you have a rare and complex disease like PSC. All of our information is checked by PSC experts and supported by evidence and it is free and easy to access - no registration required.
This is our PSC Awareness Day 2020 video
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What is the Cause of PSC?
PSC is a complex liver disease and we don’t fully understand what causes it
Current evidence suggests PSC is caused by a combination of multiple genetic changes in our DNA and so far unknown environmental triggers making an individual prone to immune attack 13. This means that people with a particular genetic makeup may be sensitive to some sort of environmental trigger that causes their immune system to ‘attack’ their bile ducts. We’re not sure what that trigger is. It could be diet, exposure to toxins, infection, microbiota (the bugs that live inside us) or even a combination of all these factors 14.
There is currently more research investigating our condition than ever before, so we’re hopeful that one day we will understand the exact cause of the disease. Understanding the cause of PSC and how it develops may help scientists to define strategies to interrupt that process and treat PSC. It is possible that drugs already available for other diseases could one day be used to treat PSC 2. This is called ‘drug repurposing’.
Variants of PSC
PSC is a complex and varied condition with different ways of presenting itself. These different forms of PSC are called ‘subtypes’ or ‘variants’. A number of forms of PSC have been proposed because of differences between patients based on the location of bile duct damage, having inflammatory bowel disease or not, different immunology on blood testing and even risk factors for progression of the disease 6,7.
There are three well-established subtypes of PSC 8:
- Classical or large duct PSC
- Small duct PSC
- PSC with features of autoimmune hepatitis (AIH), also known as 'overlap' syndrome
Other types of PSC include:
- PSC with no IBD
- Recurrent PSC (rPSC)