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Type 1 Autoimmune Hepatitis Mimic Cryptogenic Hepatitis

疑似不明原因肝炎之自體免疫性肝炎

摘要


自體免疫性肝炎在慢性活動性肝炎中雖不常見,但為一個可治療的重要疾患。在台灣,大部分的臨床醫生熟悉病毒性肝炎,而易於忽略其他少見的原因。本文報告一典型的自體免疫性肝炎。五十一歲女性於近半年來呈現間歇性黃疸,曾住院經包含肝生檢的一系列檢查後,診斷為不明原因之慢性肝炎。我們發覺病人的免疫球蛋白G為正常值的兩倍,抗核抗體及抗粒腺體抗體為陰性,而抗平滑肌抗體為陽性(l:40),肝生檢呈現為介面性肝炎(interface hepatitis)。根據一九九九年 修訂的診斷標凖符合“確定的自體免及性肝炎”(definite autoimmune hepatitis)之診斷。病人使用口服類固醇治療後,在持續追蹤呈現良好療效。因為缺乏特定的單一檢驗方法來診斷自體免疫性肝炎,自體免疲性肝炎常易被忽略。由於未經治療的自體免疫性肝炎預後不佳,我們在此強調診斷的重要性,並對相關文獻做一回顧。

並列摘要


Autoimmune hepatitis is a treatable chronic necroinflammatory liver disorder, that responds well to immunosuppressive therapy, but has a poor prognosis if left untreated. It is characterized by circulating autoantibodies and a high serum immunoglobulin G level, but there is no single diagnostic test for it, and so it is easily ignored. We report a typical autoimmune hepatitis case without initial recognition. A 51 year-old female with intermittent jaundice and elevated serum aminotransferase for more than six months, who was diagnosed as cryptogenic chronic active hepatitis before, with the histological evidence of chronic hepatitis, negative viral markers, and negative antinuclear antibody. We found she had markedly elevated serum globulin level and no available data of other autoantibodies, so reconsidered autoimmune hepatitis. After serial examination of various autoantibodies, autoimmune hepatitis was diagnosed by the revised scoring system for diagnosis of autoimmune hepatitis. Patient had good response with oral prednisolone. Negative antinuclear antibody could not rule out the existence of autoimmune hepatitis, and should not be relied upon as a major screening test. We should consider autoimmune hepatitis in cases of cryptogenic chronic active hepatitis with elevated serum γ-globulin level, especially in female or those with other autoimmune disorders.

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