title: Re-analysis of clinical features of 89 patients with autoimmune hepatitis using the revised scoring system proposed by the International Autoimmune Hepatitis Group.
abstract: OBJECTIVE
  The diagnostic criteria of autoimmune hepatitis (AIH) were recently modified by the International Autoimmune Hepatitis Group. This study was performed to assess the impact of the revised scoring system on the diagnosis of AIH.
  
  
  METHODS
  We re-analyzed the clinical features of 89 patients diagnosed as AIH in Nagasaki Prefecture, Japan, using the revised scoring system, and compared the scores and final diagnosis with our previously published results using the original system.
  
  
  RESULTS
  Of the 89 patients with AIH, 40 (45%) were classified using the new system as "definite" AIH, 41 (46%) as "probable" AIH, and 8 (9%) patients were categorized as "others". Of these, 37 (42%), 35 (39%), and 4 (4%) patients who were classified as "definite", "probable", and "others" by the original system remained in the same category by the revised system, respectively. However, 3, 4, and 6 patients were re-categorized as "definite" from "probable", "others" from "probable", and "probable" from "definite", respectively. The difference in aggregate scores between the above two systems ranged from -5 to +2. The main contributing factors to the changes in aggregate AIH score were "other autoimmune disease(s)" and "interface hepatitis without lobular involvement and bridging necrosis on liver histology". However, the main contributing factors to the demotions from "definite" to "probable" and form "probable" to "others" were those related to the characteristics of biliary diseases, i.e., antimitochondrial antibody positive, biliary changes in liver histology, and alkaline phosphatase: aspartate aminotransferase ratio between 1.5 and 3.0. Moreover, two patients who had no histological evidence of AIH were both re-categorized as "others" from "probable" AIH.
  
  
  CONCLUSIONS
  Our results indicated that the diagnosis, whether based on the revised or original system, was the same in the majority of AIH patients, but the revised scoring system excluded cases who had features suggestive of biliary diseases from "definite" AIH, and also confirmed that a diagnosis of "definite" AIH should not be made without liver histology.
authors.affiliation: Second Department of Internal Medicine, Nagasaki University School of Medicine.
authors.forename: K
authors.initials: K
authors.lastname: Omagari
authors.forename: J
authors.initials: J
authors.lastname: Masuda
authors.forename: Y
authors.initials: Y
authors.lastname: Kato
authors.forename: K
authors.initials: K
authors.lastname: Nakata
authors.forename: T
authors.initials: T
authors.lastname: Kanematsu
authors.forename: Y
authors.initials: Y
authors.lastname: Kusumoto
authors.forename: I
authors.initials: I
authors.lastname: Mori
authors.forename: R
authors.initials: R
authors.lastname: Furukawa
authors.forename: H
authors.initials: H
authors.lastname: Tanioka
authors.forename: H
authors.initials: H
authors.lastname: Tajima
authors.forename: M
authors.initials: M
authors.lastname: Koga
authors.forename: M
authors.initials: M
authors.lastname: Yano
authors.forename: S
authors.initials: S
authors.lastname: Kohno
mesh_terms: D000328:Adult; D000368:Aged; D000410:Alanine Transaminase / Q000097:blood; D000428:Alcohol Drinking / Q000453:epidemiology; D000469:Alkaline Phosphatase / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D000918:Antibody Specificity; D001219:Aspartate Aminotransferases / Q000097:blood; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000276:immunology / Q000473:pathology; D001652:Bile Ducts / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D020022:Genetic Predisposition to Disease; D005838:Genotype; D006684:HLA-DR Antigens / Q000032:analysis / Q000235:genetics; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000276:immunology / Q000473:pathology; D006525:Hepatitis, Viral, Human / Q000175:diagnosis; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008108:Liver Diseases, Alcoholic / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000276:immunology; D012189:Retrospective Studies
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D006684:HLA-DR Antigens; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase