Primary sclerosing cholangitis (PSC)

It is thought that PSC is caused by the body's own immune system. In this rare liver disease, the bile ducts become inflamed before cholestasis occurs. Cholestasis can result in cirrhosis with scarring of the liver. An additional complication of PSC is bile duct cancer. In 60% to 80% of cases, PSC is accompanied by an inflammatory bowel disease (examples: Crohn's disease, ulcerative colitis).

Symptoms

The possible symptoms include yellowing of the skin and the whites of the eyes (jaundice) deficiencies in fat-soluble vitamins A, D, E, and K, in addition to severe itching and fatty stools.

Diagnosis

Blood tests commonly show elevated levels of alkaline phosphatase and gamma-GT. However, these values do not confirm PSC. Some autoantibodies (ANA, SMA and particularly pANCA) may be present in PSC, but are not necessarily evidence of the presence of PSC.

Specific for the diagnosis is the examination of the bile ducts inside and outside the liver. The imaging technique chosen for this purpose is, on the one hand, non-invasive MRCP (magnetic resonance cholangiopancreaticography). The other option is the more invasive ERCP (endoscopic retrograde cholangiopancreaticography). If an intervention on the bile ducts (e.g. tissue sampling) is already planned, ERCP can be combined with it and makes perfect sense.

A needle biopsy of the liver can provide information about the damage to the liver already present, but is not the first choice as a diagnostic tool.

Treatment

In order to improve the elevated liver values, PSC patients take medicines like ursodeoxycholic acid (UCDA).

Severe cholestasis can be addressed with an ERCP procedure. The narrow points in the bile ducts can be widened during this procedure. UDCA and ERCP together provide a good treatment that increases life expectancy and quality.

Symptoms like itching or bile duct infections can be treated with medicines and vitamin supplements can be used to correct the deficiencies.

If PSC has developed to the advanced cirrhosis stage, only a liver transplant will help.

Primary sclerosing cholangitis (PSC): Interview with Prof. Dr. med. Stephan Vavricka

Primary sclerosing cholangitis (PSC): Interview with Prof. Dr. med. Stephan Vavricka

“What is primary sclerosing cholangitis?” Prof. Vavricka addresses in this interview PSC, one of the rare so-called autoimmune liver diseases. Bile duct cancer can occur as an additional complication of PSC. He explains that in 60% to 80% of cases, PSC is accompanied by inflammatory bowel disease (examples: Crohn’s disease, ulcerative colitis). More information about…

Primary sclerosing cholangitis (PSC): Presentation by Prof. Dr. med. Stephan Vavricka

Primary sclerosing cholangitis (PSC): Presentation by Prof. Dr. med. Stephan Vavricka

“What does primary sclerosing cholangitis mean?” PSC is probably caused by the patient’s own immune system, explains Prof. Vavricka. In this rare liver disease, the bile ducts first become inflamed before a bile stasis occurs. The bile stasis can lead to cirrhosis as the liver becomes scarred. More information about the liver disease Primary sclerosing…

Primary sclerosing cholangitis (PSC): Interview with patient Elias

Primary sclerosing cholangitis (PSC): Interview with patient Elias

“What means PSC to you?” is one of the questions in this interview with patient Elias about primary sclerosing cholangitis (PSC). PSC is probably caused by the patient’s own immune system. In this video, you will learn how Elias copes with his liver disease and how it shapes his life. More information about the liver…