Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, Milan 20090, Italy; Liver Unit, Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano 20089, Milan, Italy.
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, Milan 20090, Italy.
Clin Liver Dis. 2018 Aug;22(3):429-441. doi: 10.1016/j.cld.2018.03.001.
Primary biliary cholangitis (PBC) is considered a model autoimmune disease, characterized by circulating anti-mitochondrial antibodies and a selective autoimmune destruction of intrahepatic cholangiocytes. PBC is heterogeneous in its presentation, symptomatology, disease progression, and response to therapy. The pathogenesis is still largely unknown and epidemiologic studies have facilitated the identification of risk factors and the understanding of disease prevalence, geographic variations, heterogeneity, and differences in sex ratio. Recent studies from large international cohorts have better identified prognostic factors suggesting a change in patient management based on risk-stratification tools to identify subgroups at greatest potential benefit from second-line therapies.
原发性胆汁性胆管炎（PBC）被认为是一种自身免疫性疾病模型，其特征是循环抗线粒体抗体和肝内胆管细胞的选择性自身免疫破坏。PBC 在其表现、症状、疾病进展和对治疗的反应方面存在异质性。发病机制在很大程度上尚不清楚，流行病学研究有助于确定危险因素，并了解疾病的流行率、地理差异、异质性和性别比例差异。来自大型国际队列的最新研究更好地确定了预后因素，这表明基于风险分层工具来识别最有可能从二线治疗中获益的亚组，从而改变患者管理。
