title: Clinical and pathological characteristics of the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome.
abstract: OBJECTIVE
  The defining of the autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) overlap syndrome as a separate clinicopathological entity has been controversial and temporally and geographically subjective.
  
  
  METHODS
  From 1979 until 2000, 227 patients diagnosed with AIH, PBC or the overlap thereof were treated. Cases with genuine AIH/PBC overlap syndrome were sorted out using close clinical follow up and serial liver biopsies.
  
  
  RESULTS
  Of the 227 patients, 19 (8.4%) were diagnosed with the AIH/PBC overlap syndrome. They all cleared a score >10 for the diagnosis of AIH, and tested positive for antimitochondrial antibodies during their courses. Long-term follow up with frequent histological examinations, however, established the diagnosis of AIH/PBC overlap syndrome in only two (0.8%) patients. The most powerful factor distinguishing AIH from PBC was acidophilic bodies in lobules that were detected significantly more frequently in patients with AIH than PBC or spurious overlap syndrome (39/46 [85%]vs 3/85 [4%], P < 0.001). It was more reliable than bile-duct lesions for the distinction of PBC from AIH.
  
  
  CONCLUSIONS
  Although AIH/PBC overlap syndrome does exist, it is infrequent and needs to be diagnosed carefully using close clinical and histological follow up to enable timely and effective treatment.
authors.affiliation: Department of Gastroenterology, Toranomon Hospital, Tokyo, Japan. suzunari@interlink.or.jp
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Yasuji
authors.initials: Y
authors.lastname: Arase
authors.forename: Kenji
authors.initials: K
authors.lastname: Ikeda
authors.forename: Satoshi
authors.initials: S
authors.lastname: Saitoh
authors.forename: Akihito
authors.initials: A
authors.lastname: Tsubota
authors.forename: Fumitaka
authors.initials: F
authors.lastname: Suzuki
authors.forename: Masahiro
authors.initials: M
authors.lastname: Kobayashi
authors.forename: Norio
authors.initials: N
authors.lastname: Akuta
authors.forename: Takashi
authors.initials: T
authors.lastname: Someya
authors.forename: Yuzo
authors.initials: Y
authors.lastname: Miyakawa
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Kumada
mesh_terms: D000328:Adult; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D013577:Syndrome