Department of human pathology, Kanazawa University Graduate School of Medicine, Kanazawa 920-8640, Japan.
Clin Res Hepatol Gastroenterol. 2011 May;35(5):347-52. doi: 10.1016/j.clinre.2011.01.018. Epub 2011 Apr 6.
Pathological features of primary biliary cirrhosis (PBC) are reviewed. Immune-mediated, non-suppurative cholangitis is the initial lesion and is followed by the gradual and extensive destruction of bile ducts and development of chronic cholestasis. Simultaneously, necro-inflammatory activities of the hepatic parenchyma and limiting plates of milder form develop not infrequently. Eventually, liver fibrosis and cirrhosis develop. A new system applicable to needle liver biopsies in which staging is evaluated using a combination of three factors (fibrosis, cholestasis, and bile duct loss) and necro-inflammatory activities of the bile duct and hepatic parenchyma are graded, is proposed. The clinical and therapeutic evaluation of PBC using this system is warranted.
原发性胆汁性肝硬化（PBC）的病理特征如下。免疫介导的非化脓性胆管炎是初始病变，随后逐渐广泛地破坏胆管并发展为慢性胆汁淤积。同时，肝脏实质和较轻微形式的限制板的坏死性炎症活动也经常发生。最终，肝纤维化和肝硬化发展。提出了一种新的适用于针吸肝活检的系统，该系统使用组合三种因素（纤维化、胆汁淤积和胆管丢失）以及胆管和肝实质的坏死性炎症活动来评估分期，并对其进行分级。使用该系统对 PBC 进行临床和治疗评估是合理的。
