Primary sclerosing cholangitis – the Norwegian experience

Abstract

Research related to primary sclerosing cholangitis (PSC) has since 1980 been a major activity at the Oslo University Hospital Rikshospitalet. The purpose of this publication is to describe the development of this research, the impact of this research on the clinical handling of the patients, and finally to describe what we believe are the most urgent, remaining problems to be solved. During the early years, our research dealt primarily with clinical aspects of the disease. The concomitant inflammatory bowel disease (IBD) seen in most patients with PSC was a major interest and we also started looking into genetic associations of PSC. Prognosis, malignancy development and treatment with special emphasis on transplantation have later been dealt with. These activities has had impact on several aspects of PSC management; when and how to diagnose PSC and variant forms of PSC, how to handle IBD in PSC and how to deal with the increased rate of malignancy? The problems remaining to be solved are many. What is the role of the gut and the gut microbiota in the development of PSC? Do the PSC patients have an underlying disturbance in the bile homeostasis? And how does the characteristic type of fibrosis in PSC develop? The genetic studies have supported a role for the adaptive immune system in the disease development, but how should this be dealt with? Importantly, the development of malignancy in PSC is still not understood, and we lack appropriate medical treatment for our patients.

Key Words:

• Biliary-clinical
• cholangiocarcinoma
• inflammatory bowel disease
• liver transplantation
• primary sclerosing cholangitis

Acknowledgements

We thank the large number of present and previous collaborators, in Norway and internationally, for their continuous support and inspiration. Friends and colleagues at Oslo University Hospital Rikshospitalet, Haukeland University Hospital and within the International PSC Study Group (www.ipscsg.org) need to be mentioned in particular. We also want to underscore the paramount importance for the Scandinavian PSC research deriving from efforts laid down by Ulrika Broomé at Huddinge in Stockholm, who passed away in 2006. The Norwegian PSC research center is supported by a grant from Canica A/S.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.