Primary sclerosing cholangitis (PSC): condition overview and infographic
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What is primary sclerosing cholangitis (PSC)?
Primary sclerosing cholangitis (PSC) is a rare chronic liver disease, in which the bile ducts (both intra-and extra-hepatic bile ducts)1 —the tubes that carry bile from the liver to the small intestine—become narrowed and can harden (sclerosis) from inflammation and scarring (fibrosis). Over time the scar tissue can build-up and block parts of the bile duct completely. The accumulation of bile can gradually damage the liver and result in cirrhosis or liver failure.
Bile is a yellow-brown or green fluid produced in the liver and stored in the gallbladder, and aids digestion.
PSC affects 1:10,0002
PSC more commonly affects men than women
PSC is generally diagnosed between the ages of 25 to 60, although it can occur at any age
What causes PSC?
PSC is an autoinflammatory disease. It is unclear what triggers the inflammation of the bile ducts, some people may have a higher genetic risk, and those with existing autoimmune conditions may also be more likely to be affected by PSC.
About 70% to 80% of people with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis.3
Symptoms of PSC
Many people will initially have no symptoms and PSC and is often diagnosed through routine blood tests that show abnormal liver results. Symptoms can include:
fatigue: the most common symptom and often considered to be the most life-limiting
severe itching (pruritus): often on the palms of the hands and soles of the feet
jaundice: yellowing of the skin and the whites of the eyes
abdominal pain: often in the upper right hand part of the abdomen
fever and chills: which may indicate a bacterial infection in the bile ducts
depression and anxiety: the impact of living with PSC on quality of life can lead to depression and anxiety
Treatment of PSC
There is currently no cure or proven treatment to slow the progression of PSC. Care is focused on regular monitoring and management of symptoms. Regular scans check for changes and complications, and monitor for liver and gallbladder cancer for those with a more advanced stage of the disease, to aid early detection and treatment.
There are procedures to open blocked ducts, while liver transplant is the only known cure for advanced disease or liver failure.
References
[1] https://bestpractice.bmj.com/topics/en-gb/847
[2] https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797
[3] https://www.cuh.nhs.uk/patient-information/primary-sclerosing-cholangitis-psc
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