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酷似急性病毒性肝炎的自身免疫性肝炎急性加重——病例系列及文献综述

Acute Exacerbation to Autoimmune Hepatitis Mimicking Acute Viral Hepatitis-A Case Series and Review of Literature.

作者信息

Taneja Sunil, Kumar Pramod, Mitra Suvradeep, Duseja Ajay, Minz Ranjana, Das Ashim, Dhiman Radha K, Chawla Yogesh

机构信息

Department of Hepatology, Post Graduate Institute of Medical Education & Research, Chandigarh, India.

Department of Histopathology, Post Graduate Institute of Medical Education & Research, Chandigarh, India.

出版信息

J Clin Exp Hepatol. 2018 Mar;8(1):98-103. doi: 10.1016/j.jceh.2017.08.005. Epub 2017 Sep 1.

Abstract

BACKGROUND

Acute exacerbation of Autoimmune Hepatitis (AIH) poses a significant challenge for diagnosis as it can mimic acute viral hepatitis especially in absence of autoantibodies and hypergammaglobulinemia.

AIM

To determine the clinical, laboratory, histopathological characteristics and response to treatment in AIH patients with acute exacerbation.

METHODS

A retrospective analysis of 16 patients with acute exacerbation of AIH diagnosed over a period of eight years (2008-2016).

RESULTS

Out of the 111 patients diagnosed with AIH, acute exacerbation of AIH was diagnosed in 16 (14.4%) patients. All patients were females with median age of 35 years. Nine patients (56%) had Type 1 AIH and seven (44%) patients were diagnosed with seronegative AIH. All 16 (100%) patients had acute viral hepatitis like illness at presentation. The median bilirubin was 4.2 mg/dl (range, 2.2-20), aspartate transaminase was 568 IU/L (range, 390-908), alanine transaminase was 430 IU/L (range, 257-1026) and serum alkaline phosphatase was 395 IU/L (range, 112-890) during symptomatic period. The histopathological examination showed underlying chronic hepatitis in 10 (71.4%) patients, only fibrosis in 2 (14.2) patients and cirrhosis with activity in 2 (14.2%). All 16 (100%) patients were treated with a combination of steroids and azathioprine. Thirteen (81%) patients achieved complete biochemical remission and three (19%) patients achieved partial remission out of which one (6%) patient succumbed to illness because of the complications of cirrhosis.

CONCLUSION

A suspicion of acute exacerbation of AIH should be considered in patients with unexplained acute hepatitis mimicking acute viral hepatitis in the absence of positive viral markers. Through evaluation with immunoserological markers and liver biopsy can clinch the diagnosis of acute exacerbation of AIH in such cases.

摘要

背景

自身免疫性肝炎(AIH)急性加重对诊断构成重大挑战,因为它可酷似急性病毒性肝炎,尤其是在缺乏自身抗体和高球蛋白血症的情况下。

目的

确定AIH急性加重患者的临床、实验室、组织病理学特征及治疗反应。

方法

对8年(2008 - 2016年)期间诊断为AIH急性加重的16例患者进行回顾性分析。

结果

在111例诊断为AIH的患者中,16例(14.4%)诊断为AIH急性加重。所有患者均为女性,中位年龄35岁。9例(56%)为1型AIH,7例(44%)诊断为血清阴性AIH。所有16例(100%)患者就诊时均有急性病毒性肝炎样疾病表现。症状期时,中位胆红素为4.2mg/dl(范围2.2 - 20),天冬氨酸转氨酶为568IU/L(范围390 - 908),丙氨酸转氨酶为430IU/L(范围257 - 1026),血清碱性磷酸酶为395IU/L(范围112 - 890)。组织病理学检查显示,10例(71.4%)患者存在潜在慢性肝炎,2例(14.2%)仅有纤维化,2例(14.2%)为活动性肝硬化。所有16例(100%)患者均接受了类固醇和硫唑嘌呤联合治疗。13例(81%)患者实现了完全生化缓解,3例(19%)患者实现了部分缓解,其中1例(6%)患者因肝硬化并发症死亡。

结论

对于无阳性病毒标志物但酷似急性病毒性肝炎的不明原因急性肝炎患者,应考虑AIH急性加重的可能性。通过免疫血清学标志物评估和肝活检可确诊此类病例中的AIH急性加重。

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[Autoimmune hepatitis and overlap syndrome: diagnosis].[自身免疫性肝炎与重叠综合征:诊断]
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本文引用的文献

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EASL Clinical Practice Guidelines: Autoimmune hepatitis.欧洲肝脏研究学会临床实践指南:自身免疫性肝炎
J Hepatol. 2015 Oct;63(4):971-1004. doi: 10.1016/j.jhep.2015.06.030. Epub 2015 Sep 1.
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Autoimmune hepatitis--Update 2015.自身免疫性肝炎——2015年更新
J Hepatol. 2015 Apr;62(1 Suppl):S100-11. doi: 10.1016/j.jhep.2015.03.005.
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Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands.荷兰自身免疫性肝炎的流行病学及临床特征
Scand J Gastroenterol. 2014 Oct;49(10):1245-54. doi: 10.3109/00365521.2014.946083. Epub 2014 Aug 15.
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Present status of autoimmune hepatitis in Japan: a nationwide survey.日本自身免疫性肝炎的现状:一项全国性调查。
J Gastroenterol. 2011 Sep;46(9):1136-41. doi: 10.1007/s00535-011-0421-y. Epub 2011 May 20.

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