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| 1 | Primary biliary cirrhosis: Clinical and laboratory criteria for its diagnosis显示文摘Primary biliary cirrhosis(PBC) is a chronic progressive cholestatic granulomatous, and destructive inflammatory lesion of small intralobular and septal bile ducts, which is likely to be caused by an autoimmune mechanism with a the presence of serum antimitochondrial antibodies and a potential tendency to progress to cirrhosis. Despite the fact that the etiology of this disease has beenunknown so far, there has been a considerable body of scientific evidence that can reveal the clinical and laboratory signs of PBC and the individual components of its pathogenesis and elaborate diagnostic criteria for the disease and its symptomatic therapy. Deficiencies in autoimmune tolerance are critical factors for the initiation and perpetuation of the disease. The purpose of this review is to summarize the data available in the literature and the author's findings on clinical and laboratory criteria for the diagnosis of PBC. This review describes the major clinical manifestations of the disease and the mechanisms of its development. It presents the immunological, biochemical, and morphological signs of PBC and their significance for its diagnosis. A great deal of novel scientific evidence for the problem of PBC has been accumulated. However, the inadequate efficiency of therapy for the disease lends impetus to the quest for its etiological factors and to further investigations of its pathogenetic mechanisms and, on this basis, to searches for new methods for its early diagnosis. | Vasiliy Ivanovich Reshetnyak | 2015 | World Journal of Gastroenterology2015，21，25： | 19 |
| 2 | 亚洲地区原发性胆汁性肝硬化的诊疗现状显示文摘 | 范雪 贾继东 | 2010 | 临床肝胆病杂志2010，26，4： | 10 |
| 3 | Concept on the pathogenesis and treatment of primary biliarycirrhosis显示文摘Primary biliary cirrhosis (PBC) is an organ-specific autoimmune disease that predominantly affects women and is characterized by chronic, progressive destruction of small intrahepatic bile ducts with portal inflammation and ultimately fibrosis, leading to liver failure in the absence of treatment. Little is known about the etiology of PBC. PBC is characterized by anti-mitochondrial antibodies and destruction of intrahepatic bile ducts. The serologic hallmark of PBC is the presence of auto-antibodies to mitochondria, especially to the E2 component of the pyruvate dehydrogenase complex (PDC). Current theories on the pathogenesis of PBC favor the hypothesis that the disease develops as a result of an inappropriate immune response following stimulation by an environmental or infectious agent. Some reports suggest that xenobiotics and viral infections may induce PBC. The pathogenetic mechanism is believed to be caused by a defect in immunologic tolerance, resulting in the activation and expansion of self-antigen specific T and B lymphocyte clones and the production of circulating autoantibodies in addition to a myriad of cytokines and other inflammatory mediators. This leads to ductulopenia and persistent cholestasis, by developing end-stage hepatic-cell failure. In this review are given our own and literary data about mechanisms of development of intrahepatic cholestasis and possible ways of its correction. | Vasiliy Ivanovich Reshetnyak | 2006 | World Journal of Gastroenterology2006，12，45： | 10 |
| 4 | Role of autoimmunity in primary biliary cirrhosis显示文摘Primary biliary cirrhosis(PBC) is an autoimmune liver disease characterized by the presence of serum autoantibodies and chronic nonsuppurative destructive cholangitis.The pathogenesis of PBC involves environmental factors,genetic predisposition and loss of immune tolerance.In recent years,it has become univocally accepted that an inappropriately activated immune response is one of the most important factors in PBC.In this study,the role of autoimmunity in PBC is summarized and a feasible research orientation is recommended. | Tian-Yan Shi Feng-Chun Zhang | 2012 | World Journal of Gastroenterology2012，18，48： | 8 |
| 5 | 自身免疫性胆管炎—抗线粒体抗体阴性原发性胆汁性胆管炎还是独立的疾病?显示文摘自身免疫性胆管炎(AIC)首次被报道于1987年,是一种慢性胆汁淤积性疾病,好发于中年女性,与原发性胆汁性胆管炎(PBC)有共同的临床表现、生物化学异常及病理学改变。但血清抗线粒体抗体(AMA)阴性,抗核抗体和/或平滑肌抗体阳性率较高。治疗上对熊去氧胆酸、类固醇的反应差,需加用免疫抑制剂治疗,且预后不佳。目前关于AIC确切病理机制尚不清楚,对其分类为新的一种自身免疫性肝病或AMA阴性PBC尚无统一论断。现通过检索归纳国内外有关AIC的文章进行分类总结,并与PBC进行对比。 | 钱建丹 王贵强 | 2019 | 中华肝脏病杂志2019，27，5： | 7 |
| 6 | IgM在原发性胆汁性肝硬化中的免疫学意义及其临床应用显示文摘原发性胆汁性肝硬化(PBC)是一种慢性进展性自身免疫性肝病,目前认为其发生机制主要是由于机体免疫系统对位于胆管上皮细胞线粒体上的自身抗原发生异常的免疫应答,从而引起免疫介导的胆管损伤和汇管区炎症。抗线粒体抗体阳性、血清IgM水平升高和汇管区淋巴细胞浸润是其主要的免疫学特征,而对IgM与PBC的基础和临床研究是阐明其异常免疫应答机制的一个重要途径。现将PBC患者高IgM水平的发生机制以及IgM在PBC中的免疫学作用及其临床应用概述如下,以IgM为切人点的研究也将有助于PBc的诊断和治疗。中华检验医学杂志,2013,36:201-205) | 黄珊珊 马雄 | 2013 | 中华检验医学杂志2013，36，3： | 4 |
| 7 | Primary biliary cholangitis:Diagnosis and treatment显示文摘Primary biliary cholangitis(PBC)is an autoimmune disease that causes progressive destruction of the intrahepatic bile ducts.Although the pathogenesis of PBC is not completely understood,it is characterized by cholestatic inflammation resulting in a spectrum of cholangitis,varying degrees of fibrosis,and,potentially,cirrhosis.PBC affects all races;however,there have been few clinical studies of the disease in Asian countries,particularly in Southeast Asia,suggesting that PBC may be underreported in the Asian population.Here,we review the epidemiology,natural history,predictive and diagnostic features,and current and emerging treatments for PBC.Our goal is also to highlight current studies from Asian countries to provide a better understanding of PBC in this patient population. | Michael Chew Christopher L.Bowlus | 2018 | Liver Research2018，2，2： | 0 |
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