title: Current pharmacotherapy for cholestatic liver disease.
abstract: BACKGROUND
  The cholestatic liver diseases comprise a heterogeneous group of disorders which, left untreated, usually progresses to cirrhosis and liver failure. Most are recognized before the onset of advanced fibrosis, thereby affording an opportunity for disease modifying therapy.
  
  
  METHODS
  This review will cover the current pharmacologic management of the most common causes of cholestatic liver disease in adults, including primary biliary cirrhosis, primary biliary cirrhosis-autoimmune hepatitis overlap syndrome, primary sclerosing cholangitis, intrahepatic cholestasis of pregnancy, intestinal failure-associated liver disease, and immunoglobulin G4-associated cholangitis. Pharmacologic management of complications of cholestasis will also be reviewed.
  
  
  CONCLUSIONS
  Effective therapy for most cholestatic liver disease is lacking. Ursodeoxycholic acid (UDCA) slows the progression of primary biliary cirrhosis but the majority of patients do not have a full response. Even in those with a complete response, UDCA does not cure the disease. There is currently no effective medical therapy for primary sclerosing cholangitis. Symptoms and serum liver biochemistry values in intrahepatic cholestasis of pregnancy are improved with UDCA, but it is not certain if this alters the course of disease. Immunoglobulin G4-associated cholangitis is responsive to steroids but may relapse. The farnesoid X receptor agonists are a promising new class of drugs currently being tested in cholestatic liver disease.
authors.affiliation: Mayo Clinic in Arizona, Division of Hepatology, Phoenix, AZ 85054, USA. carey.elizabeth@mayo.edu
authors.forename: Elizabeth J
authors.initials: EJ
authors.lastname: Carey
authors.forename: Keith D
authors.initials: KD
authors.lastname: Lindor
mesh_terms: D015209:Cholangitis, Sclerosing / Q000188:drug therapy*; D002779:Cholestasis / Q000188:drug therapy*; D058607:Fibric Acids / Q000627:therapeutic use; D019693:Hepatitis, Autoimmune / Q000188:drug therapy*; D006801:Humans; D018160:Receptors, Cytoplasmic and Nuclear / Q000819:agonists; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use; D000099150:Receptor, Farnesoid X-Activated
chemical_list: D058607:Fibric Acids; D018160:Receptors, Cytoplasmic and Nuclear; D014580:Ursodeoxycholic Acid; D000099150:Receptor, Farnesoid X-Activated