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[自身免疫性肝病]

[Autoimmune liver diseases].

作者信息

Lüth S, Weiler-Normann C, Schramm C, Lohse A W

机构信息

I. Medizinische Klinik, Universitätsklinikum Hamburg-Eppendorf, Martinistrasse 52, 24046, Hamburg, Deutschland.

出版信息

Internist (Berl). 2009 Mar;50(3):310-7. doi: 10.1007/s00108-008-2254-1.

Abstract

Autoimmune hepatitis (AIH) can occur in all age groups. AIH affects women more commonly than men (3:1). Clinical presentation may be an acute hepatitis up to fulminant liver failure, but can also be asymptomatic. AIH is characterized by lymphoplasmacellular infiltrates on liver biopsy, elevated liver enzymes in serum and the absence of active viral markers. Patients characteristically present with hypergammaglobulinemia, elevated serum levels of IgG and autoantibodies. Corticosteroids are the drug of choice for induction of remission, azathioprine the drug of choice for maintenance of remission. Rapid response to immunosuppressive treatment supports the diagnosis and leads to a good long-term prognosis.Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are immune mediated diseases affecting bile ducts. While PBC has a slow progression to cirrhosis and complications mostly will be limited to complications of cirrhosis, PSC additionally carries a high risk of developing cholangiocellular carcinoma. The treatment of choice in PBC and PSC is oral ursodeoxycholic acid which may slow progression of liver disease and may ameliorate lab findings.

摘要

自身免疫性肝炎(AIH)可发生于所有年龄组。AIH在女性中的发病率高于男性(3:1)。临床表现可能为急性肝炎直至暴发性肝衰竭,但也可能无症状。AIH的特征是肝活检显示淋巴细胞和浆细胞浸润、血清肝酶升高且无活跃的病毒标志物。患者通常表现为高球蛋白血症、血清IgG水平升高和自身抗体阳性。皮质类固醇是诱导缓解的首选药物,硫唑嘌呤是维持缓解的首选药物。对免疫抑制治疗的快速反应支持诊断,并导致良好的长期预后。原发性胆汁性肝硬化(PBC)和原发性硬化性胆管炎(PSC)是影响胆管的免疫介导性疾病。虽然PBC进展为肝硬化的速度较慢,并发症大多局限于肝硬化的并发症,但PSC还具有发生胆管细胞癌的高风险。PBC和PSC的首选治疗药物是口服熊去氧胆酸,它可能会减缓肝病进展并改善实验室检查结果。

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