Objective First we intend to study further the pathogenesis of PSC. Thereby we may beable to better understand how to treat these patients medically. Secondly wewant to study the prognosis (and prognostic factors) in PSC, which isurgently needed in order to pick the right patients for transplantation. Itis also vital to identify factors of importance for development ofcholangiocarcinoma. Our third aim is therefore to identify patients likelyto develop CC before this development takes place.Primary sclerosing cholangitis (PSC) is a rare disorder although it seems tobe a difference in incidence between north and south of Europe. Mostpatients have concomitant inflammatory bowel disease. There is apreponderance of male patients and the age of debut is around 30 years. Itseems to be a genetic predisposition to develop PSC, although the geneconveying the disease susceptibility has not been identified. Nor has beenestablished an effective medical treatment. PSC has therefore become a majorindication for hepatic transplantation. Five joint studies have beenplanned. In study I the importance of neutrophilic autoantibodies in thepathogenesis and outcome of PSC will be dealt with. In study II thepathogenetic importance of a newly discovered 40 kD protein in PSC will beexamined. In two studies we will try to identify factors of importance forthe development of end stage disease (study III) and CC (study IV) amongpatients with PSC with and without CC. The participants of this jointproject have already established The European Study Group of PSC. We haveplanned to meet annually at one of the large European Congresses to deliverour results. We believe the project will provide a gradual increase ofknowledge that in the course of few years will be of high clinical interest. Fields of science medical and health sciencesclinical medicinegastroenterologyinflammatory bowel diseasenatural sciencesbiological sciencesbiochemistrybiomoleculesproteinsmedical and health sciencesclinical medicinetransplantation Programme(s) FP4-BIOMED 2 - Specific research, technological development and demonstration programme in the field of biomedicine and health, 1994-1998 Topic(s) 4.6.1 - Basic and clinical research in rare diseases Call for proposal Data not available Funding Scheme CON - Coordination of research actions Coordinator Rijkshospitalet Oslo Address 32,pilestredet 0027 Oslo Norway See on map EU contribution € 0,00
