title: [Clinical and pathological features of primary biliary cirrhotic patients with negative anti-mitochondria antibody].
abstract: OBJECTIVE
  To explore the clinical and pathological features of primary biliary cirrhosis (PBC) patients with negative anti-mitochondria antibody (AMA).
  
  
  METHODS
  Two hundreds and eight PBC patients were enrolled. The clinical and histological data of the negative AMA cases were compared with the AMA/AMA-M2 positive cases.
  
  
  RESULTS
  30 out of the 208 cases (14.4%) were AMA negative patients in our study. The general status, biochemical tests and histological findings between the two groups had no significant difference (P > 0.05). The Gamma-globulin, IgG, IgM and IgA levels of AMA/AMA-M2 positive PBC patients were higher than that of the AMA negative cases (P < 0.05). The abnormal rate of cholesterol in AMA negative PBC patients was 65.4% as compared to 50.4% in AMA/AMA-M2 positive cases, no significant difference existed between (P > 0.05). Anti-nuclear antibody (ANA) was observed in 29 (96.7%) AMA negative PBC patients, including 14 (48.3%) with granular pattern, 8 (27.6%) with nuclear membrane pattern, 6 (20.7%) with kinetochore pattern and 1 (3.4%) with homogeneous pattern. AMA negative PBC patients had elevated serum ALP, GGT, IgM and cholesterol levels, and decreased serum AST, IgG and IgA levels as compared with that of autoimmune hepatitis patients (P < 0.05, respectively).
  
  
  CONCLUSIONS
  In cholestatic patients with elevated IgM and cholesterol levels, ANA positive with non-homogeneous pattern, the diagnosis of PBC should be suspected, albeit AMA negative. The clinical, biochemical and histological features of the AMA negative PBC patients were similar to classic PBC patients, but quite different from autoimmune hepatitis.
authors.affiliation: Department of Gastroenterology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai Institute of Digestive Disease, Shanghai 200001, China.
authors.forename: Qi-xia
authors.initials: QX
authors.lastname: Wang
authors.forename: Lei
authors.initials: L
authors.lastname: Shen
authors.forename: Xiao-yu
authors.initials: XY
authors.lastname: Chen
authors.forename: De-kai
authors.initials: DK
authors.lastname: Qiu
authors.forename: Xiong
authors.initials: X
authors.lastname: Ma
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000276:immunology* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D005719:gamma-Globulins / Q000378:metabolism
chemical_list: D000974:Antibodies, Antinuclear; D005719:gamma-Globulins