Autoimmune hepatitis in a demographically isolated area of Australia
Autoimmune hepatitis in a demographically isolated area of Australia
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DOI：
10.1111/j.1445-5994.2009.02041.x
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发表时间：
2010-04-01
影响因子：
2.1
通讯作者：
Thomson, A.
中科院分区：
文献类型：
--
作者：
Haider, A. S.;Kaye, G.;Thomson, A.
背景：先前描述自身免疫性肝炎（AIH）的研究来自肝移植中心，其中病例的倾斜分布可能会对AIH的发病率及其自然史产生误导。本系列报告描述了澳大利亚首都直辖区（ACT）及周边地区稳定且人口统计学上离散的AIH患者。方法：记录42例1型AIH患者的临床、实验室和组织学特征，对初始治疗的反应，维持治疗的细节和结果。结果：与其他出版物一致，男女比例为1:3，就诊时平均年龄为53岁，24%诊断时患有肝硬化。大多数患者（86%）对初始治疗有反应，67%进入长期缓解期。一名患者死于肝功能衰竭，没有人需要肝移植。74%的病例将硫唑嘌呤纳入治疗方案，剂量一般< 2mg /kg。硫唑嘌呤剂量大于或等于2mg /kg与较好的临床结果相关，但没有达到统计学意义。女性患者出现肝硬化的比例较高（9/10 vs 1/10; P = 0.24）。结论：在澳大利亚的一项社区研究中，1型AIH主要是一种晚期疾病，对常规免疫抑制治疗有反应，通常预后良好。有必要进一步研究硫唑嘌呤的使用，以确定最佳剂量。
Background:Previous studies describing autoimmune hepatitis (AIH) come from liver transplant centres in which a skewed distribution of cases may give a misleading picture of the incidence of AIH and its natural history. This series describes AIH in a stable and demographically discrete population of patients in the Australian Capital Territory (ACT) and the surrounding region.Methods:In 42 patients with type 1 AIH (point prevalence 8 per 100 000 population), clinical, laboratory and histological features at presentation, response to initial therapy, details of maintenance therapy and outcome were recorded.Results:Consistent with other publications, the male-to-female ratio was 1:3, mean age at presentation was 53 years and 24% had cirrhosis at diagnosis. Most patients (86%) responded to initial therapy and 67% went into long-term remission. One patient died from liver failure and none required liver transplantation. Azathioprine was included in the treatment regimen in 74% of cases with doses generally < 2 mg/kg. Azathioprine dose greater than or equal to 2 mg/kg was associated with better clinical outcome, but this did not reach statistical significance. A higher proportion of female patients had cirrhosis at presentation (9/10 vs 1/10; P = 0.24).Conclusion:In this Australian community-based study, type 1 AIH was primarily a disease of later life, responded to conventional immunosuppressive therapy and generally has a good prognosis. Further study of the use of azathioprine is warranted to determine the optimal dose.