Abstract
Primary sclerosing cholangitis (PSC) is a progressive auto-inflammatory condition of the biliary ducts clinically characterized by painless cholestasis and jaundice. Histologically, the typical findings in PSC are periductal fibrosis with inflammation, bile duct proliferation, and ductopenia. These hallmarks eventually develop into end-stage liver disease requiring liver transplantation (LT), although the latency between diagnosis and LT is variable among patients. PSC is the leading indication for LT among patients with autoimmune liver disease. The interplay of PSC and inflammatory bowel disease (IBD) is intricate and poorly understood, as exemplified by the ongoing debate as to whether these are 2 distinct diseases or a complex 2-sided manifestation of the same disease spectrum. A true pathophysiological pathway has not been pinpointed, which explains the current lack of disease-specific therapies approved for this entity. This review summarizes our current knowledge about the epidemiology, pathophysiology, clinical presentation and management of PSC. We will also elucidate the relationship between PSC and IBD, specifically regarding the LT and pouchitis subpopulations.