Department of Gastroenterology and Hepatology, University Hospital, University of Duisburg Essen, Essen, Germany.
Department of Gastroenterology and Hepatology, University Hospital, University of Magdeburg, Magdeburg, Germany.
Digestion. 2018;98(2):104-111. doi: 10.1159/000487940. Epub 2018 Apr 26.
BACKGROUND/AIMS: Autoimmune hepatitis (AIH) is a relatively rare cause of liver dysfunction and may lead in some cases to acute liver failure (ALF). The aim of our study was to evaluate the clinical course and outcome of patients with AIH-induced ALF.
We retrospectively enrolled 32 patients with AIH-induced ALF and 93 age- and sex-matched patients with chronic AIH (cAIH) who were enrolled at the University Clinic Essen from 1988 to 2014. All ALF patients were treated with corticosteroids after diagnosis.
Overweight, higher γ-globulin levels, the absence of anti-smooth muscle antibodies and human leukocyte antigen (HLA) B8 and the presence of anti-mitochondrial antibodies and HLA DR7 were risk factors for an ALF vs chronic hepatitis manifestation of AIH. Liver histology was significantly more often typical for AIH in an ALF setting than in cAIH. The spontaneous survival rate was 91% and 97% in ALF and cAIH patients, respectively, at 6 months after diagnosis and only 1 patient in the ALF group developed sepsis under therapy.
Liver biopsy in an AIH-mediated ALF setting was both safe and effective in diagnosing AIH. Corticosteroid therapy was not associated with high mortality or sepsis. Our findings suggest that corticosteroid treatment of AIH-mediated ALF may improve the outcome.
背景/目的：自身免疫性肝炎（AIH）是一种相对罕见的肝功能障碍病因，在某些情况下可能导致急性肝衰竭（ALF）。我们的研究目的是评估 AIH 诱导的 ALF 患者的临床过程和结局。
我们回顾性纳入了 32 名 AIH 诱导的 ALF 患者和 93 名年龄和性别匹配的慢性 AIH（cAIH）患者，这些患者于 1988 年至 2014 年在埃森大学诊所就诊。所有 ALF 患者在诊断后均接受皮质类固醇治疗。
超重、γ-球蛋白水平较高、缺乏抗平滑肌抗体和人类白细胞抗原（HLA）B8，以及存在抗线粒体抗体和 HLA DR7，是 AIH 发生 ALF 与慢性肝炎表现的危险因素。与 cAIH 相比，ALF 时肝组织学更常表现为典型的 AIH。诊断后 6 个月，ALF 和 cAIH 患者的自发存活率分别为 91%和 97%，仅有 1 例 ALF 患者在治疗期间发生脓毒症。
在 AIH 介导的 ALF 中进行肝活检在诊断 AIH 方面既安全又有效。皮质类固醇治疗与高死亡率或脓毒症无关。我们的研究结果表明，皮质类固醇治疗 AIH 介导的 ALF 可能改善结局。
