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What is Primary Sclerosing Cholangitis (PSC)?
Primary Sclerosing Cholangitis (PSC) is a rare condition that affects the biliary structures within the body, both within and outside of the liver. The biliary structures are key to the transport of bile, a substance that is manufactured in the liver and is important in digestion. In PSC, the biliary tree becomes inflamed and damaged overtime stopping the normal flow of bile through the liver and to the digestive tract. This to fibrosis of the biliary ducts and also of the liver itself.
Primary Sclerosing Cholangitis should not be confused with the similarly named Primary Biliary Cholangitis (PBC). Whilst the two conditions do share some similar features, they are ultimately quite different and require different management plans.
Who typically gets PSC?
As we are not yet sure exactly what causes PSC, it is hard to say who the typical sufferer is. Current theory generally centres around a genetic cause. However, it does tend to be diagnosed in adulthood and affects more men than women.
What are the symptoms of PSC?
The biliary structures, which become narrowed (called stricturing) and damaged in PSC, help transport bile to where it is required to aid digestion. Therefore, when they are damaged, vitamin deficiency is a common outcome. Your doctor may use the word Cholestasis to refer to this obstructed bile flow due to PSC.
Other symptoms may include fatigue and abdomen pain in the first instance, but this can progress to itching, jaundice and ultimately liver failure. The majority of PSC sufferers also suffer from irritable bowel syndrome (IBS).
How is PSC diagnosed?
PSC is currently generally diagnosed via a combination of bloods, imaging and biopsy. A blood test would look particularly at markers which can indicate the health of the biliary system such as ALP (Alkaline Phosphatase), but there will be many other blood based indicators that a liver blood test will be able to measure.
In addition to this, many sufferers have an MRI scan of their biliary tree called an MRCP (Magnetic Resonance Cholangiopancreatography). Finally, biopsy, where a small sample of affected tissue is taken, is an option for both diagnosis and monitoring of the condition.
What is the treatment for PSC?
There is currently no known cure for Primary Sclerosing Cholangitis, and therefore treatment is more a question of management. Your doctor may prescribe you drugs which aid in symptoms such as itching. It is possible you may also be given a drug called Ursodeoxycholic Acid which from early study is thought to possibly slow progression of the condition. Ultimately, many sufferers require liver transplant.
Are there any PSC support groups I can contact?
PSC Support, is a leading patient organization for PSC, offering support and comprehensive information to patients and healthcare professionals.maintain a full information website and support structures for sufferers of PSC.
PSC Partners is a US based organisation who offer similar support.