Two sister cases of
autoimmune hepatitis are described. Case 1 involved a 49-year-old woman who suffered from
bleeding gums and general
fatigue. Her laboratory data showed a marked increase in
transaminase levels, an elevated
IgG level with titers 1:80 or more of both antinuclear and smooth muscle
antibodies and
thrombocytopenia. Histology of the biopsied liver revealed
chronic active hepatitis with a moderate infiltration of mononuclear cells. A complication of
idiopathic thrombocytopenic purpura was determined based on higher titers of PA-
IgG and a normal bone marrow findings. Case 2 involved a 54-year-old woman, an elder sister of case 1, who suffered from general
fatigue with
jaundice. Her laboratory data showed a severe damage of liver function and an elevated
IgG level with positive
antibodies to nuclear and smooth muscle
antigen. Histology of the biopsied liver revealed
chronic active hepatitis. Both patients were negative to markers of hepatotrophic agents. Under diagnosis of
autoimmune hepatitis, they have been treated with
prednisone followed by a significant clinical improvement. HLA types of two patients were Bw54-DR4 and DR4. Among 4 other siblings, the eldest sister suffered from
rheumatoid arthritis. The occurrence of two sister cases of type-1
autoimmune hepatitis has rarely reported and the fact would support a role of enviromental factors besides genetic factors for the onset of this disease.