Inflammatory Bowel Disease and Primary Sclerosing Cholangitis

Inflammatory bowel disease (IBD) is a chronic condition which is characterized by recurrent immune-mediated inflammation of the gastrointestinal system. IBD is frequently associated with extraintestinal manifestations (EIM) characterized by involvement of multiple organs. EIM occur in 21% to 47% of IBD patients (Navaneethan & Shen, 2010). While some extraintestinal manifestations are encountered more frequently in Crohn’s disease (CD) than in ulcerative colitis (UC), some are encountered equally in CD and UC. While the degree of involvement of the skin, eyes, and joints is parallel to disease activity, hepatobiliary and pulmonary involvement is independent of disease activity and intestinal inflammation (Greenstein AJ, et al., 1996). Hepatopancreatobiliary (HPB) manifestations are the most frequently encountered EIM in patients with IBD. They can be encountered in various ways: 1. HPB conditions sharing the same pathological mechanisms with IBD (Primary Sclerosing Cholangitis (PSC), small-duct PSC/pericholangitis, and PSC/autoimmune hepatitis overlap, IBD associated acute or chronic idiopathic pancreatitis) 2. HPB conditions that reflect the degree of pathophysiologic damage seen in IBD (Cholelithiasis and portal vein thrombosis) 3. HPB conditions related to side effects of drugs used in treatment of IBD (Drug induced or associated hepatitis, pancreatitis, cirrhosis, Hepatitis B reactivation, hepatosplenic T cell lymphoma) 4. HPB conditions possibly related to IBD (Autoimmune pancreatitis, IgG4-associated cholangitis, fatty liver, hepatic amiloidosis, granulomatous hepatitis, primary biliary cirrhosis) (Navaneethan & Shen, 2010) The various HPB manifestations and their associations are summarized in Tables 1 and 2. The aim of this chapter is to cover in detail primary sclerosing cholangitis, which is an important and frequently encountered HPB manifestation of ulcerative colitis.