Lammers Willem J, Kowdley Kris V, van Buuren Henk R
Dept. of Gastroenterology and Hepatology, Erasmus University Medical Centre, Rotterdam, The Netherlands.
Liver Center of Excellence, Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA, USA.
Ann Hepatol. 2014 Jul-Aug;13(4):316-26.
Primary biliary cirrhosis (PBC) is a slowly progressive autoimmune liver disease that may ultimately result in liver failure and premature death. Predicting outcome is of key importance in clinical management and an essential requirement for patients counselling and timing of diagnostic and therapeutic interventions. The following factors are associated with progressive disease and worse outcome: young age at diagnosis, male gender, histological presence of cirrhosis, accelerated marked uctopenia in relation to the amount of fibrosis, high serum bilirubin, low serum albumin levels, high serum alkaline phosphatase levels, esophageal varices, hepatocellular carcinoma (HCC) and lack of biochemical response to ursodeoxycholic acid (UDCA). The prognostic significance of symptoms at diagnosis is uncertain. UDCA therapy and liver transplantation have a significant beneficial effect on the outcome of the disease. The Mayo risk score in PBC can be used for estimating individual prognosis. The Newcastle Varices in PBC Score may be a useful clinical tool to predict the risk for development of esophageal varices. Male gender, cirrhosis and non-response to UDCA therapy in particular, are risk factors for development of HCC.
原发性胆汁性肝硬化（PBC）是一种缓慢进展的自身免疫性肝病，最终可能导致肝衰竭和过早死亡。预测疾病转归在临床管理中至关重要，也是患者咨询以及诊断和治疗干预时机选择的一项基本要求。以下因素与疾病进展及较差的转归相关：诊断时年龄较轻、男性、组织学上存在肝硬化、相对于纤维化程度而言显著的胆管减少加速、血清胆红素水平高、血清白蛋白水平低、血清碱性磷酸酶水平高、食管静脉曲张、肝细胞癌（HCC）以及对熊去氧胆酸（UDCA）无生化反应。诊断时症状的预后意义尚不确定。UDCA治疗和肝移植对疾病转归有显著的有益作用。PBC的梅奥风险评分可用于评估个体预后。PBC的纽卡斯尔静脉曲张评分可能是预测食管静脉曲张发生风险的一种有用的临床工具。特别是男性、肝硬化以及对UDCA治疗无反应，是发生HCC的危险因素。
