目的:分析自身免疫性肝病(ALD)临床特征及实验室检测结果,为进一步ALD的诊断和治疗提供理论依据.方法:对321例ALD患者的临床及实验室检查资料进行回顾性分析,比较自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)、原发性硬化性胆管炎(PSC)及自身免疫重叠综合征(OLS)的临床表现、自身抗体的检测、病理特点等.结果:(1)ALD临床表现特点各异,以皮肤瘙痒、肝功异常较多;(2)自身抗体检测:汉、维、其他民族(包括哈萨克、蒙古、回族等)ANA阳性率比较,AIH组差异均无统计学意义(P>0.05),PBC组差异有统计学意义(P<0.05),其他民族(哈萨克、蒙古、回族)ANA阳性率高于汉、维族(70.58%vs40%,47.82%).AMA阳性检测,AIH、PBC组差异均无统计学意义(P>0.05),SMA阳性检测,AIH、PBC组差异均无统计学意义(P>0.05);(3)病理组织:共有96例行肝穿刺病理活检,病理结果显示75.72%(75/96)符合诊断.结论:ALD早期诊断率低,而且常常表现不典型,往往要密切联系临床表现、血液生化及自身抗体检测结果等才能作出正确诊断. Objective: To analyze the clinical features and laboratory test results of autoimmune liver disease (ALD) and provide theoretical basis for the further diagnosis and treatment of ALD.Methods: The clinical and laboratory data of 321 patients with ALD were retrospectively analyzed to compare their own The clinical manifestations of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune overlap syndrome (OLS), the detection of autoantibodies and pathological features. Results: (1) The clinical manifestations of ALD were different, with pruritus and abnormal liver function. (2) The detection of autoantibodies: The positive rates of ANA in Han, Victoria and other ethnic groups including Kazakh, Mongolian and Hui nationality, (P <0.05). The positive rates of ANA in other ethnic groups (Kazakh, Mongolian and Hui nationality) were higher than those in Han and Uygur nationality (70.58% vs 40%, 47.82 %). There was no significant difference between AMA positive group, AIH group and PBC group (P> 0.05), SMA positive group, AIH group and PBC group (P> 0.05). (3) 96 cases of liver biopsy, biopsy showed that 75.72% (75/96) consistent with the diagnosis.Conclusion: Early diagnosis of ALD Low rate, and often atypical, often in close contact with clinical manifestations, blood biochemical and autoantibodies test results to make the correct diagnosis.