Department of Medicine and Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
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Primary sclerosing cholangitis is a chronic and progressive liver disorder. The disease is characterized by progressive inflammation leading to stricturing and fibrosis of the medium and large biliary ducts in intra and/or extrahepatic ductal system. Primary sclerosing cholangitis is more common in men. It is classically associated with inflammatory bowel disease, specifically ulcerative colitis. The disease is progressive resulting in complications, including cholestasis, malignancy and hepatic failure, necessitating a liver transplantation. The median survival following diagnosis is 10–12 years, but shorter when disease is advanced at the time of diagnosis. Approximately 50% of patients meet the criteria for liver transplantation within 10–15 years of becoming symptomatic. Unfortunately, primary sclerosing cholangitis can recur after transplantation. Herein we present a review for the clinicians of this fascinating and difficult disease.
Therapies are still lacking to cure the disease or to halt its progression. Further studies are necessary to answer these questions.