线粒体抗体阴性原发性胆汁性肝硬化的自身抗体筛选及应用研究

原发性胆汁性肝硬化（primary biliary cirrhosis, PBC）是一类以肝内小胆管非化脓性改变为主要特征的自身免疫性肝病。PBC患者实验室诊断主要依赖抗线粒体抗体（anti-mitochondrial antibody, AMA）检测。AMA阴性的PBC患者则需通过肝脏病理活检方能确诊。由于后者具有一定的风险，难以为患者和医生接受。为寻找AMA阴性PBC患者诊断相关的自身抗体标志物，本次研究将应用蛋白质组学研究技术，抗原抗体复合物富集捕获与高效液相色谱-质谱联用技术，筛选相关标志物，再而验证这些自身抗体的应用于临床的效能。本项目的开展将有助于提高AMA阴性的PBC患者早期确诊率，实现患者早期治疗并提高其生活质量，为深入认识PBC提供新思路。

Primary biliary cirrhosis (PBC) is a progressive autoimmune liver disease characterized by the destruction of intra-hepatic small bile ducts. Laboratory diagnosis of PBC is mainly depended on detection of antimitochondrial antibody. PBC patients with negative AMA are diagnosed only if confirmed by liver biopsy. Since biopsy is risky, it's not so accepted by both doctors and patients. In order to explore a diagnosis related biomarker for PBC patients with negative AMA, proteomic technology, enrichment of antigen-antibody complex combining with high performance liquid chromatography - mass spectrometry technology were applied in this research. We firstly screen the possible autoantibodies for PBC patients with negative AMA, and then we verify the clinical performance of this biomarker. We believe this research will help to comprehensively promote the early diagnosis and treatment of PBC patients with negative AMA, improve patients' quality of life, and provide new threads for in-depth understanding of PBC.