title: Transitions between variant forms of primary biliary cirrhosis during long-term follow-up.
abstract: BACKGROUND
  Conditions exhibiting features of two different autoimmune liver diseases are designated overlap syndromes. Variant forms display some, but not all, characteristics of a distinct autoimmune liver disease. We describe transitions over time between variant forms of PBC, i.e. AMA-negative PBC, autoimmune hepatitis (AIH)-PBC overlap and autoimmune cholangitis (AIC) in a large cohort of PBC patients in Sweden.
  
  
  METHODS
  We retrieved all patients with variant forms of PBC in six university hospitals in Sweden, covering 60% of the Swedish population. The diagnosis of PBC and its variants was based on laboratory findings and compatible histological features. The revised autoimmune hepatitis scoring system proposed by the International Autoimmune Hepatitis Group was used to establish the diagnosis of AIH.
  
  
  RESULTS
  In a population of 800 patients with PBC, we identified 35 (5%) variant forms; 25 patients with AIH-PBC overlap, 8 with AIC and 2 with AMA-negative PBC at the time of our study. The initial diagnoses were PBC (3 patients), AIH (3), AIH-PBC overlap (16), AIC (8) and AMA-negative PBC with (1) or without (4) concomitant AIH. The median follow-up was 125 (41-360) months. Immunosuppression and ursodeoxycholic acid induced a complete or good regression of increased aminotransferases in about half of the patients who were given one or both of these treatments.
  
  
  CONCLUSIONS
  Variant forms of PBC are seen in approximately 5% of PBC patients in Sweden. Transition between different forms may occur, emphasizing the value of repeat biopsies, but established overlapping AIH-PBC seems to be stable over time.
authors.affiliation: Department of Medicine, University Hospital MAS, Malmö, Sweden. Stefan.Lindgren@med.lu.se
authors.forename: Stefan
authors.initials: S
authors.lastname: Lindgren
authors.forename: Hans
authors.initials: H
authors.lastname: Glaumann
authors.forename: Sven
authors.initials: S
authors.lastname: Almer
authors.forename: Annika
authors.initials: A
authors.lastname: Bergquist
authors.forename: Einar
authors.initials: E
authors.lastname: Björnsson
authors.forename: Ulrika
authors.initials: U
authors.lastname: Broomé
authors.forename: Ake
authors.initials: A
authors.lastname: Danielsson
authors.forename: Barbro
authors.initials: B
authors.lastname: Lebrun
authors.forename: Hanne
authors.initials: H
authors.lastname: Prytz
authors.forename: Rolf
authors.initials: R
authors.lastname: Olsson
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D001706:Biopsy; D002761:Cholangitis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents