Background/Aims: It has been reported that severe cryptogenic chronic
hepatitis may be a subgroup of autoimmune hepatitis, The aims of this
study were to investigate the clinical features, liver function tests,
human leukocyte antigens and response to immunosuppressive therapy in
severe cryptogenic chronic hepatitis, and to compare the findings in
such patients with those in patients with autoimmune hepatitis, Method
s: History of alcohol and hepatotoxic drug intake, markers of metaboli
c liver disease, autoantibodies (antinuclear antibody, smooth muscle a
ntibody, antibody to liver/kidney microsome type 1), and viral markers
(HBsAg, HBV DNA, anti-HCV, HCV RNA) were negative in all severe crypt
ogenic chronic hepatitis patients (histological activity index >9 and
alanine aminotransferase level >2xnormal), Fifteen cryptogenic patient
s (13 women; mean age, 33+/-16 years) and seven autoimmune patients (s
even women; mean age, 28+/-3.9 years; five type 1; two type 2a) receiv
ed prednisolone and azathioprine for at least 2 years. Results: Crypto
genic chronic hepatitis patients were similar to patients with autoimm
une hepatitis with respect to age, sex, clinical presentation, liver f
unction tests and Knodell scores at admission, HLA phenotype frequenci
es were comparable between cryptogenic and autoimmune groups: BW6 (77%
vs, 100%), DR4 (62% vs, 57%), and HLA B8 (15% vs, 43%), The rates of
complete and partial remissions achieved during therapy were 87% vs. 5
7% and 13% vs, 29%, respectively (p>0.05). Conclusions: The clinical,
biochemical and HLA phenotypic features, and the responsiveness to imm
unosuppressive therapy in severe cryptogenic chronic hepatitis support
the idea that it may be an autoimmune liver disease similar to autoim
mune hepatitis.