Clinical Research Center for Autoimmune Liver Disease, Beijing You'an Hospital, Capital Medical University, 100069 Beijing, China.
Dig Dis Sci. 2011 Sep;56(9):2750-7. doi: 10.1007/s10620-011-1661-7. Epub 2011 Mar 16.
Primary biliary cirrhosis (PBC) is a relatively uncommon liver disease, and information on the prognosis and survival of PBC patients in mainland China is lacking. We therefore conducted a retrospective study to investigate the prognostic factors and survival in Chinese PBC patients.
Between October 2001 and May 2009, patients registered at Beijing You'an Hospital with abnormal liver function and serum positivity for antimitochondrial antibody (AMA) and/or AMA-M2 (n = 391) were screened. Patients diagnosed with PBC were identified, and their medical data were reviewed and analyzed for mortality predictors.
A total of 147 PBC patients were identified (mean age: 54 years, range: 28-81), of whom 126 (85.7%) were women. At the time of diagnosis, 119 patients (81.0%) were symptomatic, 28(19.0%) had hepatic decompensation, and no patients were asymptomatic. During a median follow-up period of 48 months (range: 2-312), 36 patients (24.5%) died or underwent liver transplantation, and 65 patients (44.2%) developed hepatic decompensation. The overall 5-year survival rate was 79%. Multivariate analysis indicated that Mayo risk score ≥6.11(P = 0.008), and serum IgG ≥ 17.20 g/l (P = 0.016) were associated with mortality.
Most Chinese PBC patients in this study were symptomatic at diagnosis and had significant mortality. Mayo risk score, and serum IgG were independent prognostic factors for survival.
原发性胆汁性肝硬化（PBC）是一种相对少见的肝脏疾病，目前缺乏中国大陆地区 PBC 患者预后和生存情况的相关信息。因此，我们进行了一项回顾性研究，以探讨中国 PBC 患者的预后因素和生存情况。
2001 年 10 月至 2009 年 5 月，我们筛选了在北京佑安医院就诊的肝功能异常且血清抗线粒体抗体（AMA）和/或 AMA-M2 阳性的患者（n=391）。确诊为 PBC 的患者被识别出来，并对其医疗数据进行回顾性分析，以确定死亡预测因素。
共发现 147 例 PBC 患者（平均年龄：54 岁，范围：28-81 岁），其中 126 例（85.7%）为女性。在诊断时，119 例（81.0%）患者有症状，28 例（19.0%）有肝功能失代偿，没有无症状患者。在中位随访时间为 48 个月（范围：2-312 个月）期间，36 例（24.5%）患者死亡或接受了肝移植，65 例（44.2%）患者发生了肝功能失代偿。总的 5 年生存率为 79%。多因素分析表明，Mayo 风险评分≥6.11（P=0.008）和血清 IgG≥17.20 g/l（P=0.016）与死亡率相关。
在本研究中，大多数中国 PBC 患者在诊断时即有症状，且死亡率较高。Mayo 风险评分和血清 IgG 是生存的独立预后因素。
