Primary Sclerosing Cholangitis  

Primary sclerosing cholangitis (PSC) is a rare condition which causes inflammation within the bile ducts of the liver. It can lead to cirrhosis of the liver or even cancer. The condition is chronic and hard treat. PSC is the cause of 10% of all liver transplants within Europe. Unfortunately, research in this area is lacking.

However, a recent study has illuminated more of the inner workings of this disease which could lead to improved research and ultimately, care.

The Study

Michael Turner is the head of the MedUni Vienna’s Division of Gastroenterology and Hepatology. His recent study, published in Hepatology, was able to successfully identify a prognostic factor from liver biopsies which had not previously been identified. The factor is able to determine endoplasmic reticulum (ER) stress both in liver cells themselves, called hepatocytes, as well as within the bile duct epithelium.

ER stress is a complex cellular response which arises from a build-up of proteins which are misfolded within the ER.

This molecular signature can serve as a stand-alone factor to identify PSC. It does not tell us anything about the degree or stage of fibrosis.

The team has determined that those with this molecular signature have impaired responses to ER Stress as well as a poorer overall prognosis. Patients with this molecular signature experience a greater incidence of complications.

The most exciting part of this study is that it could lead to new treatment options in PSC. ER Stress can be treated with pharmaceuticals. Therefore, understanding this molecular signature could lead to more therapeutic options for patients. Researchers already know that ER Stress is caused by the build-up of toxic bile acid. They are now working on pharmaceuticals which should balance these acids. Research on the subject has already commenced.

You can read more about this study and what it could mean for PSC patients here.