| 通过中国生物医学文献数据库（CBMD）中查找《中华医学杂志》中所有关于原发性胆汁性肝硬化（PBC）的病例报道，一共收集了1417例相关病例进行研究。其中92例被诊断为原发性胆汁性肝硬化／自身免疫性肝炎（PBC/AIH）重叠综合征。1314例同时患有其他自身免疫性疾病，37例为AMA阴性原发性胆汁性肝硬化（自体免疫胆道病变）。男女比例为1：11.25，绝大多数患者为中年女性。由于起病隐袭，而且没有明显的临床症状，所以早期诊断困难很大。从起病到正确诊断需要数月，甚至超过十年。临床质谱检查、肝脏生化检查、肝脏组织病理检查、免疫学检查结果及其伴随症状与西方文献所报道的情况相似。中国医院还常采用三种AMA试验（免疫荧光法、酶联免疫吸附测定和免疫印迹）诊断PBC，其诊断意义相仿。根据中国相关医学文献记载，熊去氧胆酸常用于原发性胆汁性肝硬化的治疗。45％到89.6％患者对这种治疗有短期或长期反应（或部分反应）。报道中有62例患者死亡，大多死于胃肠道出血、难治性腹水以及肝性脑病。根据１５年的随访调查，原发性胆汁性肝硬化的平均存活时间为8.3±1.1年。而无症状起病的患者的生存时间均超过十年。在所有报道的患者中，只有极少数接受了肝移植。结论：未来，应该进一步开展包括流行病学研究、免疫病理研究、早期诊断、科学的药物治疗试验、肝移植后存活时间等方面的研究，并且认真进行跟踪随访。
Primary biliary cirrhosis in China
GUANG-BI YAO
1 Jing An Qu Central Hospital, Shanghai, China
Using CBMD data-base system,Chinese medical journals were retrieved thoroughly,1417 cases could be collected and analyzed,92 patients were diagnosed as PBC/AIH overlapped syndrome,1314 patients accompanied with other auto-immune diseases,and 37 were AMA-negative PBC(autoimmune cholangiopathy).The ratio of females and males was 11.25,most patients were middle aged.Due to the insidious onset and inapparent clinical presentation,early diagnosis could not be made in early stage.The time from onset to correct diagnosis usually form months to years,even more than 10 years.The clinical spectrum,liver biochemistry profiles,liver histopathology,immunological tests and accompanied disorders were similar as reported in Western literatures.These are three different AMA tests used in China:immunofluorescent,ELISA and immune-blot test,the diagnostic values are similar.Ursodeoxycholic acid were generally used for PBC,according to the Chinese literatures,45% to 89.6% patient could achieved complete or partial response in a short or long-term means.62 patients were died in the reports,most common caused were massive GI bleeding,refractory ascites and hepatic encephalopathy.According to a 15 years followed-up study,the average survival time was 8.3±1.1 years,the patients with asymptomatic onset all alived more than 10 years.Only a few liver transplantations were performed in those patients. Conclusion: Many future researches should be done including epidemiology study, immunopathogenesis, early recognition, evidence based drug treatment trial, long-term survival after liver transplantation and followed up study, etc.
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