A 44-year-old woman has suffered from hepatic dysfunction since 1976. In 1981, the patient was noticed to have moderately abnormal levels in transaminase and biliary enzymes along with high levels of IgM.
In January of 1989, the patient was diagnosed as asymptomatic PBC, because of a presence of anti-pyruvate dehydrqgenas antibody in her serum.
In October of 1989, she noticed a sudden onset of jaundice, with an elevated values of transaminase, bilirubin, γ-globulin and IgM. At this time, the LE cell phenomenon, antinuclear antibody, anti-DNA antibody, anti-smooth muscle antibody and anti-M2 antibody were turned to be positive except anti-M4 antibody. The biopsied liver revealed the histological findings of chronic active hepatitis with inflammatory cell infiltration, piecemeal necrosis and fibrous dilatation in the portal region along with chronic non-supprative destructive cholangitis-like findings.
Based on these findings, the patient was diagnosed as asymptomatic PBC associated with AIH. An administration of prednisolone at 40 mg/day improved markedly her symptoms, hepatic function, autoantibody titers and hepatic histological findings in the following two months.
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