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Primary sclerosing cholangitis : pathogenesis, development of cholangiocarcinoma and prognosis

Objective

First we intend to study further the pathogenesis of PSC. Thereby we may be
able to better understand how to treat these patients medically. Secondly we
want to study the prognosis (and prognostic factors) in PSC, which is
urgently needed in order to pick the right patients for transplantation. It
is also vital to identify factors of importance for development of
cholangiocarcinoma. Our third aim is therefore to identify patients likely
to develop CC before this development takes place.

Primary sclerosing cholangitis (PSC) is a rare disorder although it seems to
be a difference in incidence between north and south of Europe. Most
patients have concomitant inflammatory bowel disease. There is a
preponderance of male patients and the age of debut is around 30 years. It
seems to be a genetic predisposition to develop PSC, although the gene
conveying the disease susceptibility has not been identified. Nor has been
established an effective medical treatment. PSC has therefore become a major
indication for hepatic transplantation. Five joint studies have been
planned. In study I the importance of neutrophilic autoantibodies in the
pathogenesis and outcome of PSC will be dealt with. In study II the
pathogenetic importance of a newly discovered 40 kD protein in PSC will be
examined. In two studies we will try to identify factors of importance for
the development of end stage disease (study III) and CC (study IV) among
patients with PSC with and without CC. The participants of this joint
project have already established The European Study Group of PSC. We have
planned to meet annually at one of the large European Congresses to deliver
our results. We believe the project will provide a gradual increase of
knowledge that in the course of few years will be of high clinical interest.

Funding Scheme

CON - Coordination of research actions

Coordinator

Rijkshospitalet Oslo
Address
32,Pilestredet
0027 Oslo
Norway