Liver Zonation

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by fibrosing inflammatory destruction of the intrahepatic and/or extrahepatic bile ducts [1], leading to bile stasis, hepatic fibrosis and ultimately to cirrhosis, end-stage liver disease, and need for liver transplantation (LT). The majority of cases occur in association with inflammatory bowel disease (IBD), which often precedes the development of PSC [2]. The etiology of PSC is undefined, apart from an increasing body of evidence that points to an immunologic disturbance as a component of the disease. However, PSC lacks the features of a typical autoimmune disease and responds poorly, if at all, to typical immunosuppressive therapies [3]. No effective medical therapy for halting disease progression has been identified. A median duration of 12–18 years from the time of diagnosis before patients develop end-stage liver disease has been described. Among eligible patients, LT is currently the only life-extending therapy for patients with end-stage PSC, although the disease can recur in the allografted liver and be a cause of morbidity post-transplant [4].