Clinical characteristics of Autoimmune Hepatitis

Background: Autoimmune hepatitis (AIH) is a self-perpetuating hepatocellular
inflammation of unknown cause. It is characterized by the presence of interface hepatitis on
histologic examination, hyper gammaglobulinemia, and autoantibodies in serum. This study
was performed to determine clinical and laboratory profile of AIH in Iran.
Methods and Materials: The medical records of patients with AIH from 4 gastroenterology
clinics in Tehran were retrospectively reviewed from September 1988 to May 2003. Forty-six
patients with AIH whose medical records were complete were selected for final review.
Results: Of the 46 patients, 38 (82%) were female. The median age at presentation was 24
years. All patients fell into the category of AIH type-I. Seventeen percent had associated
autoimmune diseases with diabetes type-I and autoimmune hemolytic disease being more
common. The onset was acute in 5% and chronic in 87% with the remaining 8% being
asymptomatic. The most common symptoms were icterus (59%) and fatigue (33%) and the
most common signs were splenomegaly (33%) and hepatomegaly (30%). In all cases, HBV
and HCV serum markers were negative and serum levels of ferritin and copper were within
normal limits. High serum levels of AST, total billirubin and alkaline phosphatase were
detected in 93%, 39% and 74%, respectively while hyper gammaglobulinemia was found in
67%. SMA, ANA, PANCA and AMA were positive in 50%, 37%, 4% and 13%, respectively.
Conclusions: AIH type-I is more common in Iran than in the United States and Europe and
other types are seen rarely. Younger age at presentation, less frequent acute onset disease and
lower rate of autoantibody positivity and hyper gammaglobulinemia may merit attention.