title: Clinical management of autoimmune biliary diseases.
abstract: Autoimmune biliary disease is an umbrella term that encompasses several distinct entities such as primary sclerosing cholangitis, primary biliary cirrhosis, autoimmune hepatitis and overlap syndromes. The general approach to the diagnosis of these disorders involves investigating symptomatic patients presenting with a cholestatic biochemical profile. Asymptomatic patients are often diagnosed during investigation of other accompanying or discrete diseases. The distinction between the various entities is necessary for directing clinical management in this group of patients with an underlying autoimmune pathophysiology. Goals of management comprise treating symptoms, preventing complications and suppressing the underlying pathogenetic processes. Liver transplantation plays a vital role in the management of this group of patients and has shown a dramatic improvement in outcomes. Medical therapies such as ursodeoxycholic acid have shown mixed effects with excellent outcomes in primary biliary cirrhosis and less impressive results in primary sclerosing cholangitis. In this manuscript we aim to discuss in detail the management of these autoimmune biliary disorders and describe the effects of different therapies on outcomes on the different subsets of patients.
authors.affiliation: Cholestatic Liver Diseases Study Group, Division of Gastroenterology and Hepatology Mayo Clinic, Rochester, MN, USA.
authors.forename: Mohamad H
authors.initials: MH
authors.lastname: Imam
authors.forename: Jayant A
authors.initials: JA
authors.lastname: Talwalkar
authors.forename: Keith D
authors.initials: KD
authors.lastname: Lindor
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D016031:Liver Transplantation / Q000379:methods; D017063:Outcome Assessment, Health Care; D011379:Prognosis; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid
keywords: Autoimmune hepatitis; Clinical outcomes; Immunoglobulin g4; Overlap syndromes; Primary biliary cirrhosis; Primary sclerosing cholangitis