title: Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis with unusual initial presentation as fulminant hepatic failure.
abstract: Autoimmune hepatitis and primary biliary cirrhosis are generally easy to discriminate on the basis of clinical, laboratory, and histological findings. The presence of anti-mitocondrial antibodies seropositivity and cholestatic clinical, laboratory, and/or histological features in patients with autoimmune hepatitis indicates the overlap syndrome of autoimmune hepatitis and primary biliary cirrhosis. Fulminant hepatic failure is an unusual initial form of presentation of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. We report the case of a 50-year-old woman with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome who presented with fulminant hepatic failure. Fulminant hepatic failure has a high mortality rate and may require liver transplant. Our patient revealed a good response to corticosteroid and ursodeoxycholic acid therapy. It is important to identify and distinguish autoimmune hepatitis and variant syndromes from other forms of liver disease because of response to corticosteroid therapy.
authors.affiliation: Gastroenterology Division, Meram Medical Faculty, Selcuk University, Konya, Turkey. ekayacetin@mynet.com
authors.forename: E
authors.initials: E
authors.lastname: Kayacetin
authors.forename: S
authors.initials: S
authors.lastname: Köklü
authors.forename: T
authors.initials: T
authors.lastname: Temuçin
mesh_terms: D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D017093:Liver Failure / Q000188:drug therapy / Q000209:etiology*; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D005938:Glucocorticoids; D014580:Ursodeoxycholic Acid; D011239:Prednisolone