自己免疫性溶血性貧血を合併した抗LKM-1抗体陽性自己免疫性肝炎の1例

概要

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Autoimmune hepatitis type 2 (AIH type 2), which has anti-liver-kidney-microsome type 1 (LKM 1) antibody, is a poor prognostic disease, although the administration of corticosteroid is sometimes remarkably effective. Most of the patients are found in young female and get worse so as to progress to liver cirrhosis. A 20 years-old woman with the rare combination of AIH type 2 and autoimmune hemolytic anemia (AHA) was reported. In 1982, she presented jaundice and anemia. In 1983, the level of transaminase increased. After that, hemolytic anemia and liver dysfunction had been presented repeatedly. In September 1990, again she presented liver dysfunction. The level of bililubin and transaminase increased remarkably, and she presented striking jaundice and splenomegaly. Anti-LKM 1 antibody was presented in both sera at 12 years old and at 20 years old. Following the steroid pulse therapy, the condition of a disease has improved remarkably. In laparoscopy, her liver showed liver cirrhosis. The findings confirmed the diagnosis of AIH type 2 with AHA. This report is the first case of AIH type 2 which began from childhood in Japan.
日本臨床免疫学会の論文