title: Primary sclerosing cholangitis.
abstract: Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown origin characterized by progressive inflammation, destruction, and fibrosis of the intrahepatic and extrahepatic bile ducts. The disease leads to obliteration of intrahepatic bile ducts and to biliary cirrhosis, end-stage liver disease, and portal hypertension. Primary sclerosing cholangitis commonly occurs in the presence of inflammatory bowel disease. Its exact etiology remains unknown. As a result, there is no existing effective medical management to delay or modify the progression of the disease. Ursodeoxycholic acid, the most well-studied drug for primary sclerosing cholangitis, has demonstrated promising results when used in combination with an immunosuppressant or antibiotic. To date, liver transplantation remains the only confirmed long-term treatment of primary sclerosing cholangitis, which now accounts for 6% of adult and 1% of pediatric liver transplantations in the United States. Primary sclerosing cholangitis represents an important liver disease with major morbidity and mortality.
authors: Orthopedic Trauma Unit, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA. alexgonzales@rcn.com
authors: Mariben R
authors: MR
authors: Geonzon-Gonzales
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D015984:Causality; D002423:Cause of Death; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D002758:Cholangiography; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*; D018450:Disease Progression; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000209:etiology; D008111:Liver Function Tests; D016031:Liver Transplantation; D008403:Mass Screening; D009017:Morbidity; D015995:Prevalence; D014481:United States / Q000453:epidemiology; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D000900:Anti-Bacterial Agents; D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid