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原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)是一种以肝脏为首要靶器官的慢性进展性自身免疫性疾病,患者的抗线粒体抗体(antimitochondrial antibodies,AMA)阳性率>95%,AMA攻击聚集于PBC患者胆管上皮内的丙酮酸脱氢酶E2成分,其病变主要为肝内细小胆管的进行性非化脓性破坏性炎症,有长期持续性肝内胆汁淤积,最终演变为再生结节不明显性肝硬化[1,2]。国内外大量文献报道了PBC与干燥综合征、系统性硬化病、系统性红斑狼疮、甲状腺
Primary biliary cirrhosis (PBC) is a chronic progressive autoimmune disease with the liver as its primary target organ. The positive rate of anti -mitochondrial antibodies (AMA) is> 95% Attack gathered in the PBC patients with bile duct epithelial pyruvate dehydrogenase E2 component, the lesion is mainly for non-purulent intrahepatic bile duct destructive inflammation, persistent long-term intrahepatic cholestasis, and eventually evolved into regenerative nodules Indiscriminate cirrhosis [1,2]. A large number of domestic and foreign literature reported PBC and Sjogren’s syndrome, systemic sclerosis, systemic lupus erythematosus, thyroid