Christopher L. Bowlus M.D, Clinical Liver Disease Volume 3, Issue 2, pages 34–37, February 2014
Primary sclerosing cholangitis (PSC) is a heterogeneous, idiopathic, inflammatory disorder of the bile ducts frequently associated with inflammatory bowel diseases (IBD) of the colon. The first descriptions of PSC in the 1920s described an “obliterative cholangitis” of the extrahepatic biliary tree with diffuse thickening of the wall and narrowing of the lumen. In the 1960s, radiographic cholangiograms revealed the diffuse involvement of the intrahepatic and extrahepatic biliary tree in many cases, and histologic studies noted the wide range of findings on liver biopsy, from normal to the classic concentric biliary fibrosis. The diagnosis of PSC then and now relies upon these cholangiographic features of focal biliary strictures and dilations in the absence of a secondary cause of sclerosing cholangitis . . . The full text of this article is available, along with a video presentation of the article and an interview with the author.