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印度次大陆的自身免疫性肝炎:7年经验

Autoimmune hepatitis in the Indian subcontinent: 7 years experience.

作者信息

Gupta R, Agarwal S R, Jain M, Malhotra V, Sarin S K

机构信息

Department of Gastroenterology, Govind Ballabh Pant Hospital, New Delhi, India.

出版信息

J Gastroenterol Hepatol. 2001 Oct;16(10):1144-8. doi: 10.1046/j.1440-1746.2001.02602.x.

Abstract

BACKGROUND

Autoimmune hepatitis (AIH) is presumed to be rare in India. The present prospective study was carried out to determine the prevalence, clinical, biochemical and histological profile of patients with AIH in India.

METHODS

Consecutive patients with chronic liver disease suspected to be AIH, were screened for antinuclear antibodies (ANA), antismooth muscle antibodies (ASMA), antimitochondrial antibody (AMA), and anti-liver kidney microsomal antibodies (anti-LKM-1). Serum protein electrophoresis and liver biopsy were done. Autoimmune hepatitis was diagnosed according to the International Autoimmune Hepatitis Group criteria.

RESULTS

Fifty of 1358 (3.43%) patients with chronic liver disease were diagnosed as autoimmune liver disease; 39 with AIH, two with overlap syndrome, five with primary sclerosing cholangitis, and four with primary biliary cirrhosis. Twenty-nine patients were categorized as definite AIH and 10 as probable AIH. Autoimmune hepatitis was common in females (males : females 1:3), with a mean age of 31 +/- 17 years. Patients often presented with fatigue, jaundice and anorexia. Skin lesions (58%), joint symptoms (30%), and menstrual abnormalities (26%) were not uncommon. Mildly elevated alkaline phosphatase and hyper gamma globulinemia were seen in 78 and 91% patients, respectively. Eighty percent of patients were type I AIH, while 20% of cases remained unclassified. Histopathological changes included piecemeal necrosis (100%), plasma cell infiltration (91%), rosette formation (82%), and cirrhosis (76%). Overall mortality was 25% during a mean follow up of 15.7 +/- 17.0 months.

CONCLUSIONS

Our results clearly demonstrate that: (i) AIH is not uncommon in India; and (ii) while the profile and spectrum of AIH resembles that seen in the West, Indian patients present late, often in a cirrhotic state.

摘要

背景

自身免疫性肝炎(AIH)在印度被认为较为罕见。本前瞻性研究旨在确定印度AIH患者的患病率、临床、生化及组织学特征。

方法

对怀疑为AIH的慢性肝病连续患者进行抗核抗体(ANA)、抗平滑肌抗体(ASMA)、抗线粒体抗体(AMA)及抗肝肾微粒体抗体(抗-LKM-1)筛查。进行血清蛋白电泳及肝活检。根据国际自身免疫性肝炎小组标准诊断自身免疫性肝炎。

结果

1358例慢性肝病患者中有50例(3.43%)被诊断为自身免疫性肝病;39例为AIH,2例为重叠综合征,5例为原发性硬化性胆管炎,4例为原发性胆汁性肝硬化。29例患者被归类为确诊AIH,10例为可能AIH。自身免疫性肝炎在女性中常见(男∶女为1∶3),平均年龄为31±17岁。患者常表现为乏力、黄疸和厌食。皮肤病变(58%)、关节症状(30%)及月经异常(26%)并不少见。分别有78%和91%的患者碱性磷酸酶轻度升高及γ球蛋白血症。80%的患者为Ⅰ型AIH,20%的病例未分类。组织病理学改变包括碎片状坏死(100%)、浆细胞浸润(91%)、玫瑰花结形成(82%)及肝硬化(76%)。在平均15.7±17.0个月的随访期间,总死亡率为25%。

结论

我们的结果清楚地表明:(i)AIH在印度并不少见;(ii)虽然AIH的特征和范围与西方所见相似,但印度患者就诊较晚,常处于肝硬化状态。

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