18例无症状期原发性胆汁性肝硬化诊断特点分析

背景：原发性胆汁性肝硬化（PBC）进展缓慢,从无症状至出现症状,时间可长达数年。无症状期PBC临床表现缺乏特异性,诊断较为困难。目的：总结无症状期PBC的诊断特点。方法：纳入18例无症状期PBC患者,检测并分析其一般情况、临床、生化、病毒学、免疫学、影像学和组织学表现。结果：18例无症状期PBC患者中男2例,女16例,确诊时平均年龄47.8岁。无临床和生化异常,仅抗线粒体抗体M2亚型（AMA-M2）阳性者9例;因肝功能异常,主要是碱性磷酸酶（ALP）、γ-谷氨酰转肽酶（GGT）升高而就诊者9例,确诊时间平均8.9个月。11例（61.1%）患者AMA-M2阳性,3例gp210抗体阳性,2例SP100抗体阳性。所有患者肝穿刺病理学检查均为Ι、Ⅱ期PBC表现。结论：无症状期PBC女性多见,可仅表现为AMA-M2和（或）gp210、SP100抗体阳性,以及ALP/GGT升高。对中老年人群特别是女性,筛查肝功能和自身免疫性肝病抗体以及完善肝脏组织学检查有助于早期诊断PBC。

Diagnostic Characteristics of Asymptomatic Primary Biliary Cirrhosis:Analysis of 18 Cases

Background： Primary biliary cirrhosis （PBC） is a slow-progressive disease. Many years may elapse before asymptomatic PBC patients becoming symptomatic. The diagnosis of asymptomatic PBC is difficult due to lack of specific clinical manifestations. Aims： To analyze the diagnostic characteristics of asymptomatic PBC. Methods： Eighteen asymptomatic PBC patients were enrolled in this study. Their general status,clinical manifestations,biochemical,virological and immunological changes,as well as imaging and histological findings were assessed and analyzed. Results： Among the 18 asymptomatic PBC patients,2 were males and 16 females; the mean age when diagnosed was 47.8 years. Nine patients had only positive antimitochondrial antibody M2 subtype （AMA-M2） with no clinical and biochemical abnormalities; 9 patients had elevated alkaline phosphatase （ALP） and γ-glutamyltransferase （GGT） at their first visit,the mean time interval between first visit to establishing diagnosis was 8.9 months. Eleven patients （61.1%） had positive AMA-M2,3 were anti-gp210-positive and 2 were anti-SP100-positive. The histological features of all patients were stage Ⅰor Ⅱ PBC. Conclusions： Asymptomatic PBC most frequently affects females. Positive AMA-M2 and/or gp210,SP100 antibodies as well as elevation of ALP and GGT may be the only presenting features. Screening liver function,liver-specific autoimmune antibodies and liver biopsy histology in middle-aged and aged persons,especially females,may be helpful for the early diagnosis of PBC.