目的： 探讨不明原因胆汁淤积性肝病患者的病理学病因分布情况。方法： 收集2017年12月至2021年11月安徽地区7所医院收治的139例胆汁淤积起病的不明原因肝功能异常患者的个人信息、临床资料、实验室检查及肝穿刺病理结果,对观察指标进行统计学分析,探明胆汁淤积性肝病患者病理析因的主要分布情况及肝脏穿刺的诊断价值。结果： 139例以胆汁淤积为主要表现的不明原因肝功能异常患者中女性105例,男性34例,平均年龄（50.2±11.5）岁。所有患者全部进行肝穿刺病理学检查,结合临床资料最后明确诊断121例（87.1%）,其中自身免疫性肝病85例（61.2%）,包括原发性胆汁性胆管炎56例（40.3%）,自身免疫性肝炎15例（10.8%）,自身免疫性肝炎合并原发性胆汁性胆管炎11例（7.9%）;药物性肝损伤21例（15.1%）;代谢性脂肪性肝病5例（3.6%）;胆管消失综合征2例（1.4%）,病因暂不明确18例（12.9%）。自身免疫性肝病组患者的碱性磷酸酶（alkaline phosphatase,ALP）水平明显偏高[291.0（225.0,387.0） U/mL比236.0（181.0,293.5） U/mL,P=0.002]。结论： 胆汁淤积起病的不明原因肝损害以女性居多,疾病谱分布主要为自身免疫性肝病,尤其是原发性胆汁性胆管炎,其次为药物性肝损伤。ALP水平可能可以帮助鉴别患者是否存在自身免疫性肝病。

Objective To explore the pathology and etiology in the patients with cholestatic liver disease.Methods From December 2017 to November 2021, 139 patients with unclarified liver dysfunction manifested as cholestasis were admitted from several hospitals in Anhui province. The clinical data including personal information, laboratory examination and liver biopsy were analyzed. Results Among 139 patients, there were 105 females and 34 males, and the average age was (50.2±11.5) years. All patients underwent liver biopsy, and 121 cases (87.1%) were definitively diagnosed, while the etiology of 18 cases(12.9%) were not clear. One hundred and twenty-one patients were given one or more etiological diagnoses as follows: 85 cases (61.2%) of autoimmune liver disease, 56 cases (40.3%) of primary biliary cholangitis, 15 cases(10.8%) of autoimmune hepatitis, 11 cases(7.9%) of autoimmune hepatitis combined with primary biliary cholangitis, 21 cases (15.1%) of drug-induced liver injury, 5 cases (3.6%) of metabolic fatty liver disease and 2 cases (1.4%) of vanishing bile duct syndrome. In addition, it was found that the alkaline phosphatase(ALP) level in the patients with the autoimmune liver disease was significantly higher [291.0 (225.0, 387.0) U/mL vs. 236.0 (181.0, 293.5) U/mL, P=0.002] than that in other patients.Conclusions Most patients with liver dysfunction and cholestasis were women, and the major diagnosis was autoimmune liver disease, especially primary biliary cholangitis, and the drug-induced liver damage was also one of main causes. ALP could be used to identify whether a patient had autoimmune liver disease.