title: Identification and characterization of IgG4-associated autoimmune hepatitis.
abstract: BACKGROUND
  Autoimmune hepatitis (AIH) and autoimmune pancreatitis (AIP) share clinical and pathological features such as high serum levels of immunoglobulin (Ig) G and autoantibodies, and lymphoplasmacytic infiltration, suggesting the presence of common immunological abnormalities. However, little is known about the possible involvement of IgG4, a hallmark of AIP, in AIH.
  
  
  OBJECTIVE
  In this study, we examined whether the IgG4 response contributes to the histopathological and clinical findings in AIH.
  
  
  METHODS
  Liver sections from 26 patients with AIH, 10 patients with primary biliary cirrhosis (PBC), three patients with primary sclerosing cholangitis (PSC) and 20 chronic hepatitis patients with hepatitis C virus (HCV) infection were immunostained for IgG4. We investigated the relationship among the histopathology, the responses to steroid therapy and the IgG4 staining.
  
  
  RESULTS
  Nine of the 26 liver specimens from patients with AIH showed positive staining for IgG4 whereas none of the 10 samples from patients with PBC, the three samples from patients with PSC or the 20 samples from patients with HCV hepatitis were positive. Patients with IgG4-positive AIH also showed increased serum levels of IgG. The numbers of T cells, B cells and plasma cells were significantly increased in the livers of patients with IgG4-positive AIH as compared with those patients with IgG4-negative AIH. Patients with IgG4-positive AIH also showed a marked response to prednisolone therapy.
  
  
  CONCLUSIONS
  AIH may be classified into either an IgG4-associated type or an IgG4 non-associated type with the former showing a marked response to prednisolone treatment.
authors: Department of Gastroenterology and Hepatology, Kinki University School of Medicine, Osaka-Sayama, Osaka, Japan.
authors: Hobyung
authors: H
authors: Chung
authors: Tomohiro
authors: T
authors: Watanabe
authors: Masatoshi
authors: M
authors: Kudo
authors: Osamu
authors: O
authors: Maenishi
authors: Yoshio
authors: Y
authors: Wakatsuki
authors: Tsutomu
authors: T
authors: Chiba
mesh_terms: D000328:Adult; D000368:Aged; D015209:Cholangitis, Sclerosing / Q000276:immunology / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D019698:Hepatitis C, Chronic / Q000276:immunology / Q000473:pathology; D019693:Hepatitis, Autoimmune / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000473:pathology*; D007074:Immunoglobulin G / Q000097:blood*; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D011239:Prednisolone