Tsuneyama Koichi, Baba Hayato, Morimoto Yuki, Tsunematsu Takaaki, Ogawa Hirohisa
Department of Pathology and Laboratory Medicine, Institute of Biomedical Sciences, Tokushima University Graduate School.
J Med Invest. 2017;64(1.2):7-13. doi: 10.2152/jmi.64.7.
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease that predominantly affects middle-aged women and is characterized by the chronic progressive destruction of small intrahepatic bile ducts with portal inflammation and, ultimately, fibrosis. The serological hallmark of PBC is the presence of anti-mitochondrial autoantibodies (AMA). Several mechanisms have been proposed for immune-mediated bile duct damage in PBC, including the roles of T cells, B cells, other cell phenotypes, and AMA. A sign of fragility of biliary epithelial cells caused by apoptosis, senescence, and autophagy has also been noted. Several complex steps and mechanisms appear to be involved in the induction and progression of cholangitis and biliary degeneration in patients with PBC. J. Med. Invest. 64: 7-13, February, 2017.
原发性胆汁性胆管炎（PBC），以前称为原发性胆汁性肝硬化，是一种器官特异性自身免疫性疾病，主要影响中年女性，其特征是肝内小胆管的慢性进行性破坏伴门静脉炎症，最终导致纤维化。PBC的血清学标志是抗线粒体自身抗体（AMA）的存在。已经提出了几种PBC中免疫介导的胆管损伤机制，包括T细胞、B细胞、其他细胞表型和AMA的作用。由凋亡、衰老和自噬引起的胆管上皮细胞脆弱性迹象也已被注意到。PBC患者胆管炎和胆管变性的诱导和进展似乎涉及几个复杂的步骤和机制。《医学研究》64: 7 - 13，2017年2月。
