Zolfino T, Heneghan M A, Norris S, Harrison P M, Portmann B C, McFarlane I G
Institute of Liver Studies, King's College Hospital, London SE5 9RS, UK.
Gut. 2002 May;50(5):713-7. doi: 10.1136/gut.50.5.713.
Significant diversity in disease severity has been identified for autoimmune disorders among different ethnic groups but there is a lack of data on autoimmune hepatitis (AIH) in populations other than those of European Caucasoid (EC) or Japanese extraction.
To assess the clinical features, response to therapy, and eventual outcome in AIH patients of non-EC ethnicity.
A retrospective review of a regularly updated database of patients with AIH referred to liver outpatient clinics at King's College Hospital, London, since 1983.
Twelve patients were identified (10 female; six African, five Asian, one Arabic; median age at presentation 30 years (range 12-58)) who satisfied international criteria for type 1 (11 cases) or type 2 (one case) AIH. Nine (75%) had cholestatic serum biochemistry and three (25%) had mild biliary changes on liver biopsy without definitive features of primary biliary cirrhosis or cholangiographic evidence of primary sclerosing cholangitis. Four showed a complete biochemical response to standard prednisolone with or without azathioprine therapy, three partial, and five no response. Four have required liver transplantation for intractable disease. By comparison with 180 EC patients with definite AIH attending during the same period, the non-EC patients were younger (p<0.05), presented with cholestatic biochemistry (p=0.014), and morphological biliary features more frequently (p<0.0005) and showed a poorer initial response to standard therapy (p<0.0005).
Clinical expression of AIH in non-EC patients seems to differ in important respects from that in EC or Japanese patients. Management of such patients is challenging and may require alternative or more aggressive treatment strategies.
已发现不同种族人群中自身免疫性疾病的疾病严重程度存在显著差异，但除欧洲白种人（EC）或日本血统人群外，其他人群中自身免疫性肝炎（AIH）的数据尚缺乏。
评估非EC种族AIH患者的临床特征、对治疗的反应及最终结局。
对自1983年以来转诊至伦敦国王学院医院肝脏门诊的AIH患者的定期更新数据库进行回顾性分析。
共确定了12例患者（10例女性；6例非洲人、5例亚洲人、1例阿拉伯人；就诊时中位年龄30岁（范围12 - 58岁）），他们符合1型（11例）或2型（1例）AIH的国际标准。9例（75%）血清生化表现为胆汁淤积，3例（25%）肝活检显示轻度胆汁改变，但无原发性胆汁性肝硬化的确切特征或原发性硬化性胆管炎的胆管造影证据。4例对标准泼尼松龙治疗（联合或不联合硫唑嘌呤）有完全生化反应，3例部分反应，5例无反应。4例因难治性疾病需要肝移植。与同期就诊的180例确诊AIH的EC患者相比，非EC患者更年轻（p<0.05），血清生化表现为胆汁淤积（p=0.014），形态学胆汁特征更常见（p<0.0005），且对标准治疗的初始反应较差（p<0.0005）。
非EC患者AIH的临床表现在重要方面似乎与EC或日本患者不同。此类患者的管理具有挑战性，可能需要替代或更积极的治疗策略。
