title: Clinical and histological features of autoantibody-negative autoimmune hepatitis in Chinese patients: a single center experience.
abstract: OBJECTIVE
  This study aimed to define the clinical features of Chinese patients with autoantibody-negative autoimmune hepatitis (AIH) and to refine the diagnosis and management of these atypical patients in a single Chinese center.
  
  
  METHODS
  A retrospective evaluation of 167 Chinese patients with AIH was performed. Patients meeting comprehensive criteria with the absence of antinuclear antibodies, smooth muscle antibodies, liver-kidney microsomal-1 antibodies and antimitochondrial antibodies were defined as autoantibody-negative patients.
  
  
  RESULTS
  In total, 17 (10.2%) of the 167 patients with AIH were autoantibody-negative. The general status and biochemical tests between the classical and autoantibody-negative patients were not significantly different (P > 0.05). Serum immunoglobulin G levels of the autoantibody-negative AIH patients were lower than those of the classical AIH ones (P = 0.004). There was no significant difference in the histological inflammatory grades between the two groups; however, advanced histological stages were more common in the autoantibody-negative AIH group (P < 0.001). In the autoantibody-negative AIH patients, 11 (64.7%) had a possible diagnosis and 6 (35.3%) had a definite diagnosis according to the comprehensive criteria. While with the simplified criteria only 3 patients (17.6%) had a possible diagnosis, and none had a definite diagnosis of AIH. The complete biochemical remission rate was 86% within 24 months of immunosuppressive therapy, which was comparable to classical AIH (P = 0.658).
  
  
  CONCLUSIONS
  Autoantibody-negative AIH is not uncommon in Chinese AIH patients, and has a good response to immunosuppressive treatment comparable to classical patients.
authors.affiliation: Division of Gastroenterology and Hepatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai Institute of Digestive Diseases, Key Laboratory of Gastroenterology & Hepatology, Ministry of Health, Shanghai Jiao Tong University, Shanghai, China.
authors.forename: Qi Xia
authors.initials: QX
authors.lastname: Wang
authors.forename: Wei Jun
authors.initials: WJ
authors.lastname: Jiang
authors.forename: Qi
authors.initials: Q
authors.lastname: Miao
authors.forename: Xiao
authors.initials: X
authors.lastname: Xiao
authors.forename: Hai Yan
authors.initials: HY
authors.lastname: Zhang
authors.forename: Shan Shan
authors.initials: SS
authors.lastname: Huang
authors.forename: Lei
authors.initials: L
authors.lastname: Shen
authors.forename: Jing
authors.initials: J
authors.lastname: Hua
authors.forename: Hai
authors.initials: H
authors.lastname: Li
authors.forename: Shen
authors.initials: S
authors.lastname: Li
authors.forename: De Kai
authors.initials: DK
authors.lastname: Qiu
authors.forename: Xiong
authors.initials: X
authors.lastname: Ma
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents