Primary Biliary Cirrhosis
Primary Biliary Cirrhosis (PBC) is a relatively rare chronic liver disease with the characteristics of an autoimmune disease. The condition may lead to slowly increasing liver damage with the development of liver cirrhosis, and eventually result in liver failure and death. The name of the disease is confusing, as most patients do not show cirrhosis of the liver. The number of patients with this disease in the Netherlands is not well established; likely the number lies between 500 and 1500. The disease presents in adults only; 90% of the patients are female. The diagnosis typically is made at middle age. The disease is sometimes found more or less by coincidence and it is by far not always associated with distinct symptoms. The most frequent signs and symptoms are fatigue and itching.

Treatment
There was no effective treatment for a long time. Many drugs have been studied for their effectiveness, but it appeared that many anti-inflammatory agents and agents that affect the immune system generally are little effective or not at all. Ursodeoxycholic acid, on the other hand, is a drug that does seem effective indeed, particularly in a less advanced stage of the disease. In almost all patients administration of this bile acid leads to distinct improvement of the abnormal liver tests. On the basis of multiple studies it may be assumed as well that this has a favourable effect on the course of the disease on the long term. Ursodeoxycholic acid can be considered a natural medicine that is tolerated very well, also over a prolonged period of time. Important adverse side-effects have not been noted. For patients with severely advanced disease, liver transplantation is an effective therapy.

Current studies
A so-called cohort study is ongoing in the Netherlands since 1990, coordinated from Erasmus MC. The aim of the study is to follow patients with PBC receiving ursodeoxycholic acid treatment on the long term. Many hospitals participate in this study.

Currently the data obtained from this study are being analysed so as to ascertain the course of the disease on the long run, comparing it with the predicted course without treatment and based on data for the Dutch general population.

Analysis also involves the effect of ursodeoxycholic acid on laboratory liver test findings on the long term, as well as the significance of a certain degree of improvement in test results for the eventual outcome.

PBC occasionally co-occurs with another liver disease called autoimmune hepatitis. We are studying whether the disease course of patients with this rare overlapping clinical picture differs from that of patients who only have PBC or only autoimmune hepatitis.