title: Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis: a retrospective study of 115 cases of autoimmune liver disease.
abstract: OBJECTIVE
  The aim of this retrospective study was to compare clinical, biological, and histological features and treatment response in 115 patients with overlap syndrome (OS), autoimmune hepatitis (AIH) or primary biliary cirrhosis (PBC).
  
  
  METHODS
  Consecutive patients with AIH, PBC or OS followed between 1984 and 2005 in five different centers were included. All data were re-evaluated using current diagnostic criteria of each disease.
  
  
  RESULTS
  Fifteen patients had OS (13 females), 48 AIH (40 females) and 52 PBC (49 females). Patients with OS were significantly younger than patients with PBC (median age: 44 vs 59 years). Jaundice (20%) and pruritus (20%) were the main initial symptoms in OS. Patients with OS had serum transaminase and gammaglobulin levels significantly higher than patients with PBC; serum alkaline phosphatase, gamma-glutamyl-transpeptidase and IgM levels were significantly higher in OS than in patients with AIH. Histological analysis showed moderate or severe piecemeal necrosis in 86% and destructive cholangitis in 93% in OS group. Among 11 patients with OS treated with ursodeoxycholic acid (UDCA) or immunosuppressors alone, only 6 had a complete biochemical response. In contrast, all patients with OS receiving combined therapy, as first or second line, responded, 5 patients to the combination corticosteroids-azathioprine-UDCA and 2 to the combination cyclosporine-UDCA.
  
  
  CONCLUSIONS
  OS is not rare and accounts for 13.9% of patients with autoimmune liver disease in our series. Combination of immunosuppressors and UDCA appears the most efficient treatment in these patients.
authors.affiliation: Service d'Hépato-Gastroentérologie; CHU Reims.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Heurgué
authors.forename: Fabien
authors.initials: F
authors.lastname: Vitry
authors.forename: Marie-Danièle
authors.initials: MD
authors.lastname: Diebold
authors.forename: Nahla
authors.initials: N
authors.lastname: Yaziji
authors.forename: Brigitte
authors.initials: B
authors.lastname: Bernard-Chabert
authors.forename: Jean-Loup
authors.initials: JL
authors.lastname: Pennaforte
authors.forename: Rémi
authors.initials: R
authors.lastname: Picot
authors.forename: Hervé
authors.initials: H
authors.lastname: Louvet
authors.forename: Luc
authors.initials: L
authors.lastname: Frémond
authors.forename: Patrick
authors.initials: P
authors.lastname: Geoffroy
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Schmit
authors.forename: Guillaume
authors.initials: G
authors.lastname: Cadiot
authors.forename: Gérard
authors.initials: G
authors.lastname: Thiéfin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000188:drug therapy; D004359:Drug Therapy, Combination; D005260:Female; D005602:France; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000473:pathology*; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy*; D008111:Liver Function Tests; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid