Beijing Key Laboratory of Translational Medicine on Cirrhosis, Liver Research Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, No. 95 Yong-an Road, Xicheng District, Beijing, 100050, People's Republic of China.
Hepatol Int. 2017 Sep;11(5):412-418. doi: 10.1007/s12072-017-9819-9. Epub 2017 Sep 14.
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune liver disease characterized by progressive destruction of small intrahepatic bile ducts. If left untreated, PBC may eventually result in end-stage liver disease. For better management of PBC and optimal allocation of medical resources, it is pivotal to accurately estimate the prognosis of patients with PBC. This article will briefly review the models that predict long-term outcome of PBC patients, with special focus on the applicability, strengths and limitations of the widely used models reported from 1983 to 2016. Among many, the Mayo score has been extensively validated and considered as the classic prognostic model for untreated PBC patients, whereas the well-validated Paris I and Paris II criteria are mainly used in ursodeoxycholic acid (UDCA)-treated patients with advanced PBC (stage III-IV) and early PBC (stege I-II), respectively. Based on multicenter studies with large sample sizes, the recently reported GLOBE score and UK-PBC score seem to be superior to previous models and can be applied in patients with different stages of PBC who are already on UDCA therapy, but further external validation may be justified.
原发性胆汁性胆管炎（PBC），以前称为原发性胆汁性肝硬化，是一种自身免疫性肝病，其特征是小的肝内胆管进行性破坏。如果不治疗，PBC 可能最终导致终末期肝病。为了更好地管理 PBC 和优化医疗资源的分配，准确估计 PBC 患者的预后至关重要。本文将简要回顾预测 PBC 患者长期预后的模型，特别关注 1983 年至 2016 年报道的广泛应用的模型的适用性、优势和局限性。在众多模型中，Mayo 评分已被广泛验证，被认为是未治疗的 PBC 患者的经典预后模型，而经过良好验证的 Paris I 和 Paris II 标准主要用于晚期（III-IV 期）和早期（I-II 期）接受熊去氧胆酸（UDCA）治疗的进展期 PBC 患者。基于多中心、大样本量的研究，最近报道的 GLOBE 评分和 UK-PBC 评分似乎优于以前的模型，可适用于已接受 UDCA 治疗的不同阶段的 PBC 患者，但可能需要进一步的外部验证。
