Primary sclerosing cholangitis: best practices for diagnosis

Introduction: Primary Sclerosing Cholangitis (PSC) is a chronic disorder with cholestatic features, which is characterized by biliary strictures. The etiology is unknown but inflammation around the biliary tree leads to fibrosis which leads to both intrahepatic and in most cases also extrahepatic biliary strictures. The vast majority of patients have concomitant inflammatory bowel disease (IBD), mostly ulcerative colitis (UC) and more rarely Crohns disease (CD) Areas covered: This paper reviews the history of diagnostic strategies in PSC and the current gold standard for diagnosis of PSC. The role of liver biopsy as a part of diagnostic strategies in PSC is discussed. The differential diagnoses of PSC are reviewed and in particular causes of secondary sclerosing cholangitis are presented. Expert opinion: Patients with IBD who develop elevated liver tests with a cholestatic pattern should undergo a MRC as the suspicion of PSC is very high in this clinical situation. If the MRC demonstrates biliary strictures there is a very high likelihood that the patient has PSC if secondary sclerosing cholangitis is excluded. If the patient has characteristic biliary strictures a liver biopsy rarely adds additional information in diagnostic purposes.