title: A scoring system for primary biliary cirrhosis and its application for variant forms of autoimmune liver disease.
abstract: BACKGROUND
  Although primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) are two independent autoimmune liver diseases, it is sometimes difficult to characterize the variant forms of autoimmune liver disease. A PBC scoring system, in combination with the AIH scoring system may be helpful to characterize such patients.
  
  
  METHODS
  A PBC scoring system was introduced that selected 14 categories characteristic of PBC. One hundred and thirty-four patients with PBC, 31 patients with autoimmune cholangitis (AIC), 22 patients with overlap syndrome, and 48 patients with AIH were included in the study. The AIC patients fulfilled the PBC criteria but were negative for anti-mitochondrial antibody and positive for anti-nuclear antibody. Overlap syndrome patients fulfilled both the PBC and AIH criteria.
  
  
  RESULTS
  The total scores (means +/- SD) for the PBC, AIC, overlap syndrome, and AIH patients were 23.3 +/- 4.7, 9.3 +/- 4.4, 18.0 +/- 5.9, and 3.6 +/- 3.3, respectively. When definite and probable PBC patients were defined as those with a total score of over 17 and 9-17, respectively, all except for 1 patient could be classified as definite or probable PBC. Four of the 48 AIH patients were classified as probable PBC. PBC scores for the variant autoimmune liver diseases showed a wide deviation. Plotting both PBC and AIH scores in a rectangular coordinate enabled us to locate each patient with variant forms according to the deviation from classical PBC or AIH.
  
  
  CONCLUSIONS
  The PBC scoring system might be useful in characterizing the features of variant forms of autoimmune liver disease.
authors.affiliation: Department of Medicine and Medical Science, Okayama University Graduate School of Medicine and Dentistry, 2-5-1 Shikata-cho, Okayama 700-8558, Japan.
authors.forename: Kazuhide
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Ryo
authors.initials: R
authors.lastname: Terada
authors.forename: Ryoichi
authors.initials: R
authors.lastname: Okamoto
authors.forename: Youichi
authors.initials: Y
authors.lastname: Hiasa
authors.forename: Masanori
authors.initials: M
authors.lastname: Abe
authors.forename: Morikazu
authors.initials: M
authors.lastname: Onji
authors.forename: Takao
authors.initials: T
authors.lastname: Tsuji
mesh_terms: D001327:Autoimmune Diseases / Q000145:classification; D002761:Cholangitis / Q000145:classification; D005260:Female; D019693:Hepatitis, Autoimmune / Q000145:classification*; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000145:classification*; D008297:Male