title: Sequential presentation of primary biliary cirrhosis and autoimmune hepatitis.
abstract: BACKGROUND
  Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is used to describe the coexistence of both diseases, with either a sequential or a simultaneous presentation in the same patient. Available studies have focused on the simultaneous form, whereas there is limited information on sequential PBC-AIH. We carried out a retrospective study of patients who sequentially developed PBC-AIH overlap syndrome.
  
  
  METHODS
  The medical data of 1065 patients diagnosed with PBC (n=483) and AIH (n=582) were retrospectively analyzed.
  
  
  RESULTS
  A sequential development of PBC-AIH was observed in 19 (1.8%) patients after a mean of 6.5 (1-14) years of follow-up. AIH developed in 12 (2.5%) PBC patients, whereas PBC occurred in seven (1.2%) patients with AIH. The baseline serologic and histological findings of patients who developed PBC-AIH were similar to those of patients with typical PBC or AIH. Eighteen patients were treated with a combination of ursodeoxycholic acid (UDCA) and immunosuppression after the diagnosis of PBC-AIH was established. One patient showed a spontaneous resolution of hepatitic flare under UDCA therapy. Biochemical remission was achieved in 16 patients, whereas three progressed to decompensated cirrhosis and required liver transplantation.
  
  
  CONCLUSIONS
  The sequential overlap of PBC-AIH can occur during the follow-up of patients with pure PBC or AIH. In our cohort, we could not identify any factors that predicted the development of this rare condition. The combination of UDCA and immunosuppression seems to be an appropriate therapy in the setting of PBC-AIH.
authors.affiliation: aDepartment of Gastroenterology, Hacettepe University bDepartment of Gastroenterology, Numune Research and Education Hospital, Ankara, Turkey cDepartment of Hepato-Gastroenterology, CHU Reims, Reims dDepartment of Hepatology, Besançon, France eDepartment of Clinical Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy fDivision of Liver Diseases, the Mount Sinai Medical Center, New York, New York, USA gDepartment of Gastroenterology and Hepatology, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Cumali
authors.initials: C
authors.lastname: Efe
authors.forename: Ersan
authors.initials: E
authors.lastname: Ozaslan
authors.forename: Alexandra
authors.initials: A
authors.lastname: Heurgué-Berlot
authors.forename: Taylan
authors.initials: T
authors.lastname: Kav
authors.forename: Chiara
authors.initials: C
authors.lastname: Masi
authors.forename: Tugrul
authors.initials: T
authors.lastname: Purnak
authors.forename: Murat
authors.initials: M
authors.lastname: Torgutalp
authors.forename: Luigi
authors.initials: L
authors.lastname: Muratori
authors.forename: Solange
authors.initials: S
authors.lastname: Bresson-Hadni
authors.forename: Gérard
authors.initials: G
authors.lastname: Thiéfin
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Schiano
authors.forename: Paolo
authors.initials: P
authors.lastname: Muratori
authors.forename: Staffan
authors.initials: S
authors.lastname: Wahlin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005060:Europe; D005260:Female; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D013577:Syndrome; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D015415:Biomarkers; D005765:Gastrointestinal Agents; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid