ABSTRACT
Autoimmune liver diseases comprise a number of disorders in which inflammatory damage
to the liver is believed to derive from an autoimmune attack. These include autoimmune
hepatitis (AIH), characterised by positive smooth muscle and/or nuclear (SMA/ANA)
or liver kidney microsomal type 1 (LKM1) antibodies, autoimmune scleros-ing cholangitis
(ASC), usually SMA/ANA positive, and AIH after liver transplantation, which is positive
for SMA, ANA, or atypical LKM. These disorders often present with symptoms indistinguishable
from prolonged acute hepatitis. Less commonly the onset is insidious, with nonspecific
symptoms, or with complications of portal hypertension. For AIH and ASC, experimental
evidence suggests that usually in individuals genetically predisposed to autoimmunity,
a liver self antigenic peptide is recognized by T lymphocytes which promote a cascade
of autoaggressive processes. For AIH after liver transplantation, the pathogenic mechanisms
remain to be elucidated. All types of autoimmune liver disorders appear to respond
favourably to early treatment with prednisolone with or without azathioprine. For
patients presenting with fulminant hepatic failure or with already advanced cirrhosis,
immunosuppression is rarely effective and the only mode of treatment is liver transplantation.
The role of other immunosuppressant or immunomodulatory drugs, like cy-closporin A,
tacrolimus or ursodeoxycholic acid, in the treatment of autoimmune liver disorders
remains to be defined.
KEY WORDS
autoimmune hepatitis - sclerosing cholangitis - liver transplant - immunosuppression
