title: De novo autoimmune hepatitis after living donor liver transplantation is unlikely to be related to immunoglobulin subtype 4-related immune disease.
abstract: OBJECTIVE
  Recently, we reported that immunoglobulin subtype 4 (IgG4) is involved in autoimmune hepatobiliary diseases, such as autoimmune hepatitis, sclerosing cholangitis, and pancreatitis. However, the association of IgG4 with autoimmune hepatic disease after living donor liver transplantation (LDLT) has not been investigated.
  
  
  METHODS
  Of the 72 LDLT recipients, four patients (5.6%) were suspected of having autoimmune-related hepatic disease after LDLT. The diagnosis was made based on a histological diagnosis following an examination of a biopsy liver specimen in three cases, while in one case a pemphigoid appeared in the flank with liver fibrosis of unknown cause. Human leukocyte antigen (HLA) mismatches were 3, 2, 2, and 2, respectively. The serum level of IgG4 in the patients was measured, and IgG4 immunohistochemical staining in the liver biopsy specimens was also performed.
  
  
  RESULTS
  In all cases, steroid pulse therapy or recycle treatment and subsequent increased steroid dose as well as additional azathioprine or mycophenolate mofetil were effective. While a few positive-stained cells for IgG4 were observed in the liver of one case, negative staining for IgG4 was observed in the other cases. All serum subclasses of IgG4 were within normal limits.
  
  
  CONCLUSIONS
  In our series of LDLT, IgG4-related immune disorder is unlikely to be involved in post-transplant, autoimmune-related liver disease.
authors.affiliation: Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. sueguchi@nagasaki-u.ac.jp
authors.forename: Susumu
authors.initials: S
authors.lastname: Eguchi
authors.forename: Mitsuhisa
authors.initials: M
authors.lastname: Takatsuki
authors.forename: Masaaki
authors.initials: M
authors.lastname: Hidaka
authors.forename: Yoshitsugu
authors.initials: Y
authors.lastname: Tajima
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.forename: Yasuni
authors.initials: Y
authors.lastname: Nakanuma
authors.forename: Takashi
authors.initials: T
authors.lastname: Kanematsu
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D001379:Azathioprine / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D006680:HLA Antigens / Q000097:blood; D006505:Hepatitis / Q000188:drug therapy / Q000276:immunology*; D006650:Histocompatibility Testing; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000378:metabolism*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008099:Liver / Q000187:drug effects / Q000276:immunology*; D016031:Liver Transplantation / Q000009:adverse effects*; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives; D020551:Pulse Therapy, Drug; D013256:Steroids / Q000008:administration & dosage; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D006680:HLA Antigens; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D013256:Steroids; D009173:Mycophenolic Acid; D001379:Azathioprine