title: Centrilobular zonal necrosis as a hallmark of a distinctive subtype of autoimmune hepatitis.
abstract: OBJECTIVE
  Centrilobular zonal necrosis (CZN) is a known histological variant of autoimmune hepatitis (AIH). However, the significance of CZN is yet to be fully elucidated. This study aimed to determine whether CZN is a hallmark of a distinctive subtype of AIH.
  
  
  METHODS
  Histological changes in the centrilobular zones of liver biopsies from 113 AIH patients were assessed by a single pathologist and classified into three categories: typical zonal necrosis defined as CZN (15 patients); other necroinflammatory change (NIC; 24 patients); and absence of necrosis (non-NIC; 74 patients). The clinicopathological features and immunogenetic background of CZN patients were then assessed.
  
  
  RESULTS
  The clinicopathological features of AIH with CZN were distinct from other types of AIH, including a higher frequency of acute onset, lower frequency of antinuclear antibodies, lower antinuclear antibody titers, lower serum immunoglobulin G levels, lower grade interface hepatitis, less prominent lymphoplasmacytic infiltration, and lower AIH score. Increased and decreased frequencies of HLA-DR9 and HLA-DR4, respectively, were identified as immunogenetic features of AIH with CZN. Conversely, the clinicopathological characteristics of AIH with NIC were similar to those of non-NIC AIH, including the majority of the AIH patients. The therapeutic outcomes of AIH with CZN were excellent when precise diagnoses were made without delay.
  
  
  CONCLUSIONS
  The clinicopathological features and immunogenetic background of AIH with CZN differed from AIH without CZN. CZN may be a hallmark of a distinct subtype of AIH.
authors.affiliation: Departments of aGastroenterology and Hepatology bPathology, The Jikei University Katsushika Medical Center cDepartment of Internal Medicine, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan.
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Aizawa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Abe
authors.forename: Tomonori
authors.initials: T
authors.lastname: Sugita
authors.forename: Nobuyoshi
authors.initials: N
authors.lastname: Seki
authors.forename: Yoshimichi
authors.initials: Y
authors.lastname: Chuganji
authors.forename: Youhei
authors.initials: Y
authors.lastname: Furumoto
authors.forename: Akihiko
authors.initials: A
authors.lastname: Sakata
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D016022:Case-Control Studies; D005260:Female; D006684:HLA-DR Antigens / Q000276:immunology; D019693:Hepatitis, Autoimmune / Q000145:classification / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan; D008099:Liver / Q000187:drug effects / Q000276:immunology / Q000473:pathology*; D008103:Liver Cirrhosis / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D011237:Predictive Value of Tests; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D006684:HLA-DR Antigens; D007075:Immunoglobulin M; D007166:Immunosuppressive Agents