In children, a type of graft dysfunction associated with autoimmune feature
s has been described. We have identified 7 adult liver-transplant (LT) reci
pients from a series of over 1,000 consecutive transplant recipients who pr
esented between 0.3 years and 7.2 years following transplantation with char
acteristic symptoms, autoantibody profiles, and histologic findings of auto
immune disease. The indications for transplantation were Ecstasy overdose,
alcohol-related cirrhosis, primary sclerosing cholangitis (PSC) (2), primar
y biliary cirrhosis (PBC), hepatitis C cirrhosis, and cryptogenic cirrhosis
. Two patterns of de novo autoantibody development were noted; anti-liver-k
idney-microsome (LKM) antibody development at high titer in association wit
h an aspartate transaminase (AST) > 500 and antinuclear (ANA) and antismoot
h muscle (AMA) antibody development at titers > 1/80 with lower AST levels.
All cases had elevated IgG. Liver biopsies showed changes of an autoimmune
-type hepatitis with portal and periportal hepatitis in association with a
marked infiltrate of plasma cells, lymphocytes, and bridging collapse. Two
patients lost their grafts because of the disease. Patients were treated wi
th reintroduction of steroids and azathioprine in cases in which it had bee
n withdrawn. Major histocompatibility class I and II mismatching did not in
cur risk. Eight of 12 liver allografts were acquired from either DRB*0301-
or DRB*0401-positive donors, and 4 recipients were DRB*0301-positive. This
series illustrates that both symptoms and histologic findings of graft dysf
unction compatible with autoimmune hepatitis (AIH) exist in adult LT recipi
ents. Graft loss may be a consequence. This entity may represent a specific
type of rejection that should currently be classified as "graft dysfunctio
n mimicking autoimmune hepatitis."