目的总结国内纤维淤胆性肝炎（FCH）的临床流行病学资料及其诊疗要点。方法应用“中国生物医学文献数据库”光盘（2000年1月-2013年3月）检索FCH的文献报告，对近13年的国内纤维淤胆性肝炎的文献报告进行总结，并回顾天津市第一中心医院2000年1月-2013年3月临床病理资料，总结经病理诊断证实的纤维淤胆性肝炎的病例。总结FCH患者的临床流行病学资料、临床表现、病理诊断、治疗以及预后。结果共检索和收集到FCH病例49例，包括文献报道41例、天津市第一中心医院病理诊断8例。结果：(1)FCH与HBV或HCV感染相关；较为罕见，发生于器官移植术后等应用糖皮质激素等免疫抑制剂情况下，存在严重免疫抑制的患者；（2）FCH有独特的病理表现：肝纤维化，淤胆，肝细胞变性、坏死，炎症反应相对较轻，免疫组化证实肝细胞HBsAg或HCV阳性；（3）临床表现主要为进行性黄疸等淤胆及肝功能障碍表现；（4）该病进展迅猛，病死率极高，本组病死率为66.7%。结论在用免疫抑制剂治疗时，有HBV或HCV既往感染史的患者，应预防性抗病毒治疗；无肝炎病毒感染史的患者，应严密监测，以减少FCH发生；早期诊断、及时调整免疫抑制剂的应用、抗病毒治疗，可能改善本病预后。%Objective To summarize the clinical epidemiology data and diagnosis and treatment of ifbrosing cholestatic hepatis (FCH) reported in Chinese periodicals and diagnosed by Tianjin ifrst central hospital. Methods Articles in Chinese on FCH were screened from the Chinese Bio-Medical Database( from January 2000 to March 2013) and FCH cases ( from January 2000 to March 2013) in Tianjin ifrst central hospital were reviewed. Data of epidemiology, clinical and pathological features, treatment and prognosis of FCH were analyzed. 49 cases of FCH included 41 cases reported by other hospitals and 8 cases diagnosed by Tianjin ifrst central hospital,were selected. Results 40 cases of 49 cases were HBV recurrence, 4 cases were HCV recurrence, 4 cases were newly infected with HBV, and 1 case was newly infected with HCV. All of patients in this group were treated with immunosuppressant, such as glucocosteroid;42 cases of 49 cases were patients after organ transplantation, 7 cases of 41 cases were non-organ transplantation patients. FCH was an uncommon and rapidly progressive hepatitis, and was high mortality. The mortality of this group cases was 66.7%, and 6 cases of 7 non-organ transplantation cases died (6/7). The mean survival time was 2.2 months(11 days-7 months). The pathological features included marked hepatocytes swelling and necrosis, severe cholestasis, extensive ifbrosis, ductular reaction, and only mild inflammation. Immunohistochemistry staining shows HBsAg positive in hepatocytes. The clinic features was hyperbilirubinemia and cholestatic hepatic dysfunction. Conclusion FCH is a rapidly progressive viral hepatitis in patients with immunosuppressive treatment. FCH can suffer from post organ transplantation but also non-organ transplantation. The patients with HBV or HCV infection history and with immunosuppressant treatment are suggested preventive anti-virus therapy. Early diagnosis is very important, and active treatment is strong recommended, such as adjust the dose of immunosuppressant, antivirus therapy, support treatment etc.
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