What is PPSC

Primary Sclerosing Cholangitis (PSC)

What's this?

Primary sclerosing cholangitis (PSC) is a rare liver disease that is likely caused by one's own immune system. Initially, the bile ducts in the liver are inflamed and biliary congestion occurs. In 60 to 80% of cases, PSC is also associated with inflammatory bowel disease (Crohn's disease or ulcerative colitis). In the long term, the biliary blockage can lead to scarring of the liver (cirrhosis). Bile duct cancer can develop as an additional complication of PSC.

What are the symptoms

Possible symptoms are severe yellowing of the skin and eyes (jaundice) with severe itching, fatty stools and a lack of fat-soluble vitamins A, D, E and K.

How can you diagnose a PSC?

Overall, there are no clear blood values ​​that alone could determine or rule out PSC. Alkaline phosphatase and gamma-GT are often elevated in PSC. Various autoantibodies can also be measurable, which alone are also not conclusive for the diagnosis (ANA, SMA, in particular pANCA).

It is particularly important for the diagnosis to examine the biliary tract inside and outside the liver. In order to visualize the bile ducts, one is often first used today MRCP (Magnetic resonance cholangiopancreatography) performed. This is non-invasive and therefore has fewer risks than that ERCP (endoscopic retrograde cholangiopancreatography). The ERCP can be useful if you also want to take tissue samples or intervene therapeutically in the bile ducts. A liver puncture is less specific for PSC, but in some cases it can aid the diagnosis and determine the extent to which the liver is already scarred.

How is PSC treated?

There is currently no officially approved therapy for PSC. Some PSC patients receive ursodeoxycholic acid (UDCA) in the form of tablets. UDCA can improve elevated liver values ​​in PSC and possibly prevent bile duct cancer. Whether it has a positive overall effect on the course of the disease is controversial. The current German guideline for autoimmune liver diseases mentions UDCA as a treatment option without committing to a recommendation.

In severe biliary congestion, the affected bile ducts can be widened with an ERCP; in combination with ursodeoxycholic acid, this increases the life expectancy of the patient. Symptoms such as itching and bile duct infections are treated with their own medication if necessary. Vitamin supplements can be taken if there is a lack of vitamins.

If PSC progresses to terminal stages of advanced cirrhosis, a liver transplant is possible.

Research is currently underway on new drugs for PSC such as nor-ursodeoxycholic acid (norUDCA). Here, too, the first study results show that certain laboratory values ​​such as alkaline phosphatase can be improved as a result. The extent to which this also influences the course of the disease in the long term is currently being investigated.

How common is PSC?

PSC is a rare disease. No figures are currently known for Germany. The German guideline for autoimmune liver diseases estimates that generally around 10 out of 100,000 people in the general population are affected by PSC.

PSC affects 2.4 to 7.5% of people with ulcerative colitis and also occurs in 1.2 to 3.4% of Crohn's disease patients. According to the current guideline on autoimmune liver diseases, first-degree relatives (siblings, parents, children) of PSC patients have a 0.7% risk of also developing PSC (as of 2017).

Are PSC patients at risk of severe Covid-19 courses?

It is currently not known whether an initial PSC disease (without severe liver damage) already increases the risk of severe Covid-19 courses. The professional associations EASL and ESCMID stated in August 2020 that there was not enough meaningful data on this question.

It is known, however, that patients with cirrhosis of the liver - regardless of the cause - are more likely to have severe courses of Covid-19.

Current information on the novel Sars-CoV-2 coronavirus in liver patients can be found on our website at https://www.leberhilfe.org/coronavirus/

Can PSC patients get vaccinated against the novel coronavirus?

Deutsche Leberhilfe e.V. advocates vaccination against SARS-CoV-2 for liver patients including people with PSC, despite the unanswered questions about long-term effectiveness and safety. Hundreds of thousands of people have already been vaccinated and so far (as of December 22, 2020) we have no reports of autoimmune complications or worsening of an existing PSC. We are closely monitoring the situation. Further information on the novel corona vaccines and known side effects can be found at https://www.leberhilfe.org/coronavirus/

Who is at risk?

Patients with ulcerative colitis and Crohn's disease; Men are affected slightly more often than women (60%). Sometimes children can also be affected by PSC. First-degree relatives are also at increased risk.

Information material on PSC

You can also find written information in our 28-page PSC brochure, which you can order in our shop:
Link to the brochure "Primary Sclerosing Cholangitis"

More information on PSC

Further information can also be found at our partner association, the German Crohn's Disease and Ulcerative Colitis Association (DCCV e.V.), which has its own PSC working group:
Link to the PSC working group at DCCV e.V.
In 2017, a new German guideline on autoimmune liver diseases (AILE) was published, which also takes a position on PSC:
Link to the guideline on autoimmune liver diseases

 

Cologne | 12/2020 | Leberhilfe editorial staff