原发性胆汁性肝硬化患者肝失代偿的风险因素。
Risk factors for hepatic decompensation in patients with primary biliary cirrhosis.
机构信息
Department of Rheumatology, Key Laboratory of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 100032, China.
出版信息
World J Gastroenterol. 2013 Feb 21;19(7):1111-8. doi: 10.3748/wjg.v19.i7.1111.
AIM
To examine the clinical features and analyze prognostic factors in a prospective study of primary biliary cirrhosis (PBC) patients.
METHODS
From 1995 to 2010, PBC patients without hepatic decompensation seen at the Peking Union Medical College Hospital were enrolled. Clinical signs and manifestations (pruritus, persistent fatigue, jaundice and pain in the right hypochondrium), laboratory parameters (auto-antibodies for autoimmune hepatic disease, biliary and hepatic enzymes, immunoglobulin, bilirubin, and albumin) and imaging findings were recorded at entry and at specific time points during follow-up. Cox regression and Kaplan-Meier analyses, respectively, assessed the risk factors for hepatic decompensation and survival.
RESULTS
Two hundred and sixty-two PBC patients were enrolled with a median follow-up of 75.2 mo (range, 21-201 mo). The 240 patients were aged 51.5 ± 10.2 years at diagnosis and 91.6% were female. Two hundred and forty-five (93.5%) were seropositive for anti-mitochondrial antibodies. At presentation, 170 patients (64.9%) were symptomatic, while 96 patients (36.6%) had extra-hepatic autoimmune disease. During the follow-up period, 62 (23.7%) patients developed hepatic decompensation of whom four underwent liver transplantation and 17 died. The cumulative survival rate and median survival time were 83.9% and 181.7 mo, respectively. Cox regression analysis revealed that an incomplete ursodeoxycholic acid (UDCA) response or inconsistent treatment [P < 0.001; hazard risk (HR) 95%CI = 2.423-7.541], anti-centromere antibodies (ACA) positivity (P < 0.001; HR 95%CI = 2.516-7.137), alanine aminotransferase ratio (AAR) elevations (P < 0.001; HR 95%CI = 1.357-2.678), and histological advanced liver disease (P = 0.006; HR 95%CI = 1.481-10.847) were predictors of hepatic decompensation. The clinical features and survival of PBC in China are consistent with those described in Western countries.
CONCLUSION
Incomplete UDCA response or inconsistent treatment, ACA positivity, AAR elevations, and advanced histological stage are predictors of decompensation.
目的
在原发性胆汁性肝硬化（PBC）患者的前瞻性研究中，检查临床特征并分析预后因素。
方法
1995 年至 2010 年期间，在我院就诊的无肝功能失代偿的 PBC 患者纳入本研究。记录入组时及随访期间特定时间点的临床症状和体征（瘙痒、持续疲劳、黄疸和右季肋区疼痛）、实验室参数（自身免疫性肝病、胆汁和肝脏酶、免疫球蛋白、胆红素和白蛋白的自身抗体）和影像学发现。Cox 回归和 Kaplan-Meier 分析分别评估了肝功能失代偿和生存的危险因素。
结果
共纳入 262 例 PBC 患者，中位随访时间为 75.2 个月（范围 21-201 个月）。240 例患者在诊断时的年龄为 51.5±10.2 岁，91.6%为女性。245 例（93.5%）抗线粒体抗体阳性。在就诊时，170 例（64.9%）患者有症状，96 例（36.6%）患者有肝外自身免疫性疾病。在随访期间，62 例（23.7%）患者发生肝功能失代偿，其中 4 例接受了肝移植，17 例患者死亡。累积生存率和中位生存时间分别为 83.9%和 181.7 个月。Cox 回归分析显示，不完全熊去氧胆酸（UDCA）反应或治疗不连续[P＜0.001；风险比（HR）95%CI=2.423-7.541]、抗着丝点抗体（ACA）阳性（P＜0.001；HR 95%CI=2.516-7.137）、丙氨酸氨基转移酶比值（AAR）升高（P＜0.001；HR 95%CI=1.357-2.678）和组织学晚期肝病（P=0.006；HR 95%CI=1.481-10.847）是肝功能失代偿的预测因素。中国 PBC 的临床特征和生存情况与西方国家描述的一致。
结论
不完全 UDCA 反应或治疗不连续、ACA 阳性、AAR 升高和组织学晚期是失代偿的预测因素。
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