We retrospectively studied 42 liver biopsy specimens from 39 patients who m
et serologic and histologic criteria of autoimmune liver diseases. We found
10 cases of overlap syndrome (OLS), 10 autoimmune cholangitis (AIC), 10 pr
imary biliary cirrhosis (PBC), and 9 autoimmune hepatitis (AIH) type 1. The
following results were obtained (1) Granulomas and biliary duct lesions we
re more prominent in PBC and AIC than in OLS and AIH. (2) Bile duct loss wa
s not observed in AIH cases. (3) Features of hepatocellular damage such as
piecemeal necrosis, spotty lobular necrosis, and confluent necrosis, were m
uch more prevalent in OLS and AIH than in PBC and AIC. (4) HLA-DR antigen e
xpression by hepatocytes was more frequent in AIH and OLS, whereas the expr
ession of the same antigen by the bile duct epithelium was more frequent in
PBC and AIC. We conclude there is a morphologic spectrum in autoimmune liv
er diseases, in which PBC forms one end of the spectrum, AIH the other; OLS
the middle but closer clinically and histologically to AIH than to PBC, an
d AIC, which seems to be an antimitochondrial antibody-negative subtype of
PBC.