Development and its application of serum marker for the evaluation of staging in primary biliary cirrhosis

原发性胆汁性肝硬化（PBC）是一种慢性自身免疫性胆汁淤积性肝病，其特征是中小型胆管的破坏和传统上通过免疫荧光检测血清中抗线粒体抗体（AMA）的存在。诊断PBC的“金标准”程序是肝组织病理学检查。然而，肝活检并不是一个完全安全的程序，PBC的特征性组织病理学变化并不总是明显的活检标本。因此，血清学检查如AMA对PBC的诊断是有用的，因为这是非侵入性的，因此可以在整个疾病过程中重复进行。然而，不幸的是，到目前为止还没有“金标准”测定（即，敏感性和特异性均为100%。我们的结果表明，新开发的酶联免疫吸附试验（ELISA）的敏感性MESACUP-2测试线粒体M2试剂盒， 关于我们 高于原始ELISA，并与免疫荧光法相当。它可以特别适用于监测PBC的疾病活动和治疗结果。通过使用这些方法，可以推测日本有330，000名AMA阳性者，30，000 - 40，000名PBC患者。其中，20，000名PBC患者被诊断为患有PBC。我们的研究结果还表明，PBC和所谓的“自身免疫性胆管炎（AIC）”的诊断取决于22%的患者各自疾病的“阶段”。这可能导致在诊断AIC之前推荐Western印迹分析。AIC的明确诊断标准需要进行无选择偏倚的前瞻性研究。酶抑制法检测抗丙酮酸脱氢酶复合物（PDC）抗体具有客观、快速、简便、成本低等优点。由于这种检测方法具有几乎100%的特异性，因此它可能特别适用于筛查发展中国家的高危人群。因此，我们成功地证明了这些AMA测量的特点和利用。因此，需要开展更准确、更大规模的研究，以建立PBC的“完整”或“金标准”诊断方法。少

Primary biliary cirrhosis (PBC) is a chronic autoimmune cholestatic liver disease characterized by destruction of small and medium-sized bile ducts and the presence of antimitochondrial antibodies (AMA) in the serum traditionally detected by immunofluorescence. The "gold standard" procedure for the diagnosis of PBC is histopathological examination of liver tissue. However, liver biopsy is not a totally safe procedure, and the characteristic histopathological changes of PBC are not always evident in biopsy specimens. Therefore, serological examination such as AMA is useful for the diagnosis of PBC because this is non-invasive and therefore can be repeated throughout the course of the disease. Unfortunately, however, there is so far no "gold standard" assay (i.e., with 100% sensitivity and 100% specificity) for the detection of AMA in PBC. Our results indicated that the sensitivity of the newly developed enzyme-linked immunosorbent assay (ELISA) using MESACUP-2 Test Mitochondria M2 kit w … More as higher than that of an original ELISA, and comparable with that of immunofluorescence. It can be particularly suitable for monitoring disease activity and treatment outcome of PBC. By using these methods, it can be speculated that there are 330,000 AMA-positive people, 30,000-40,000 patients with PBC in Japan. Of these, 20,000 patients with PBC are diagnosed as having PBC. Our results also suggested that the diagnosis of PBC and so-called "autoimmune cholangitis (AIC)" was dependent on the "phase" of the respective disease in 22% of the patients. This may result in recommending Western blotting analysis before making the diagnosis of AIC. A prospective study without selection bias is needed to develop the definite diagnostic criteria of AIC. The enzyme inhibition assay for the detection of anti- pyruvate dehydrogenase complex (PDC) antibodies offers certain advantages such as objectivity, rapidity, simplicity, and low cost. Since this assay has almost 100% specificity, it may have particular applicability in screening the at-risk segment of the population in developing countries. Thus, we successfully demonstrated the characteristics and utilization of these AMA measurements. More accurate and larger study is requested for the development of a "complete" or "gold standard" diagnostic assay for PBC in the future. Less