Defining Primary Sclerosing Cholangitis: Results From an International Primary Sclerosing Cholangitis Study Group Consensus Process
Cyriel Y. Ponsioen, David N. Assis, Kirsten Muri Boberg, Christopher L. Bowlus, Mark Deneau, Douglas Thorburn, Lars Aabakken, Martti Färkkilâ, Bret T. Petersen, Christian Rupp, Stefan G. Hübscher
Abstract
Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease of the bile ducts that is surrounded by much uncertainty. First of all, it is a rare disease, with reported prevalence between 0 and 31.7 per 100,000 individuals,1,2 and establishing a diagnosis can be difficult. The primary pathology is hidden deep in the liver, and there is no specific and easy noninvasive diagnostic test for PSC. Histologic features of fibrosing cholangiopathy are present in <20% of liver biopsy specimens obtained from patients with PSC and can also be seen in cases of secondary sclerosing cholangitis.