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Autoimmune hepatitis associated with bile duct injury resembling chronic non‐suppurative destructive cholangitis
Author(s) -
Sato Yasunori,
Harada Kenichi,
Sudo Yoshiko,
Watanabe Kishichiro,
Nakahama Tohru,
Morimoto Hideo,
Nakanuma Yasuni
Publication year - 2002
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1046/j.1440-1827.2002.01377.x
Subject(s) - autoimmune hepatitis , medicine , bile duct , primary biliary cirrhosis , pathology , overlap syndrome , anti nuclear antibody , gastroenterology , intrahepatic bile ducts , hepatitis , ursodeoxycholic acid , liver biopsy , liver injury , autoantibody , antibody , biopsy , immunology , disease
Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are representative autoimmune liver diseases in which hepatocytes and intrahepatic bile ducts, respectively, are selectively damaged by autoimmune mechanisms. Bile duct injury and loss is characteristic of PBC and chronic non‐suppurative destructive cholangitis (CNSDC), in particular, is a histological hallmark of PBC. In this report, we present an unusual case of AIH accompanied by CNSDC‐like bile duct injury in a 46‐year‐old woman. The patient's serum aminotransferase level was abnormally high. The serum levels of alkaline phosphatase, γ‐GTP and IgG were also elevated, but the IgM level was within normal limits. The titer of antismooth muscle antibody (SMA) was 1:80 , while antinuclear autoantibody (ANA) and the M2 fraction of antimitochondrial antibody (AMA) were both negative. Liver biopsy disclosed CNSDC‐like bile duct injuries and severe interface hepatitis and lobular hepatitis with perivenular zonal necrosis were observed. The aggregate score of the International Autoimmune Hepatitis Group corresponded to the category of probable AIH. Immunohistochemically, histocompatibility leukocyte antigen‐DR, which is aberrantly expressed in the damaged bile ducts of PBC, was not found in the injured bile ducts of this case. Laboratory data were much improved by treatment with prednisone, but ursodeoxycholic acid was not effective. Although the possibility of an overlapping syndrome of AIH‐ and AMA‐negative PBC could not be excluded, this case was diagnosed as AIH with CNSDC‐like bile duct lesions.