The GLOBE score quantifies the risk of mortality in patients treated with ursodeoxycholic acid for primary sclerosing cholangitis. Patients with a score > 0.30 have a markedly reduced survival outside transplantation. This score makes it possible to guide the therapeutic choices to be implemented.
Primary sclerosing cholangitis is a chronic cholestatic disease with a highly variable course, characterized by inflammatory and fibrotic involvement of the intra and/or extra-hepatic bile ducts, of unknown but probably dysimmune mechanism, often associated with inflammatory colitis. The evolution is very variable but potentially serious. The two main risks are the formation of secondary biliary cirrhosis and the occurrence of cholangiocarcinoma, which is very difficult to diagnose early. In addition, in case of associated IBD, there is a high risk of colon cancer justifying special monitoring.
The diagnostic phase comprises several stages which are in fact parallel:
1) it is sclerosing cholangitis
2) this cholangitis is not secondary
3) what are the possible associated diseases?
4) what is the severity of Primary sclerosing cholangitis?
Non-invasive methods occupy a major place in the diagnosis and follow-up of Primary sclerosing cholangitis. The diagnosis of Primary sclerosing cholangitisis essentially based on imaging of the bile ducts. Thus the fibro-inflammatory nature of the biliary involvement is much more often retained on the MRI appearance than proven histologically.