Objective: To analyze the characteristics of clinical and laboratory indexes in patients with liver disease with positive anti-liver cytosol antibody type 1 (anti-LC1), in order to provide references for clinical and differential diagnosis. Methods: The clinical data of 23 832 inpatients and outpatients with positive anti-LC1 autoantibodies detected in routine autoantibody test from January 2010 to January 2020 were retrospectively analyzed, and their clinical and laboratory indexes were compared. Western blotting was used to detect anti-LC1, anti-soluble liver antigen antibody (anti-SLA), anti-glycoprotein 210 antibodies and anti-nucleosome 100 antibodies. Indirect immunofluorescence assay was used to detect anti-nuclear antibody (ANA), anti-mitochondrial antibody, anti-Smooth muscle antibody (ASMA), anti-liver and kidney microsomal antibody (anti-LKM) and other autoantibodies. Normally distributed measurement data between the two groups were compared by independent-sample t-test, and the multiple groups comparison were compared by one-way analysis of variance. Non-normally distributed measurement data were compared by non-parametric rank sum test. Results: 38 anti-LC1 positive patients were detected in 23832 autoantibody tests. The age of initial diagnosis ranged from 11.0 to 84.0 (50.6 ± 16.0) years. There were 8 males (21.1%) and 30 females (78.9%). A total of 31 cases (81.6%) were positive for anti-LC1 and ANA, and the dominant karyotype was speckled pattern, accounting for 54.8%. Five cases (13.2%) were positive for ASMA, and no simultaneous positive with anti-LKM or anti-SLA. Among the 38 anti-LC1 positive patients, 9 were diagnosed with autoimmune hepatitis (AIH), 6 with possible AIH, 6 with primary biliary cholangitis (PBC), 8 with hepatitis B, 2 with hepatitis C, 1 with alcoholic liver disease, 2 with non-alcoholic fatty liver disease, 1 with drug-induced liver injury, 1 with hepatolenticular degeneration, and 2 with tumor. Confirmed and probable AIH cases accounted for 39.5% (15/38) of anti-LC1 positive cases. Among anti-LC1 positive patients, 47.4% (18/38) had entered the stage of liver cirrhosis. AIH group globulin level was higher than HBV group (P = 0.006) and other disease groups (P = 0.001). AIH group IgG level was higher than PBC group (P = 0.027), HBV group (P = 0.009) and other disease groups (P = 0.004). the of the PBC group IgM level was higher than AIH group (P = 0.003), HBV group (P = 0.003) and other disease groups (P = 0.006). Conclusion: Anti-LC1 is not only detected in AIH, but also observed in patients with primary biliary cholangitis, hepatitis B and C, alcoholic and non-alcoholic liver disease, drug-induced liver injury, hereditary metabolic liver disease and tumor. In addition, it is mainly female gender dominance and nearly half of ANA-positive young, middle-aged and elderly patients develop liver cirrhosis. For the diagnosis of type 2 autoimmune hepatitis, whether anti-LC1 is a specific antibody needs further research, but if AIH is highly suspected, this antibody can be used as a substitute.
目的： 分析抗肝细胞溶质抗原1型抗体（抗-LC1）阳性的肝病患者临床及实验室指标特点，为临床诊断和鉴别诊断提供参考。 方法： 回顾性分析2010年1月至2020年1月住院及门诊患者中送检的包括抗-LC1 23 832例次常规自身抗体检测中检出抗-LC1阳性患者的病例资料，分析比较其临床和实验室指标。免疫印迹法检测抗-LC1、抗-可溶性肝抗原抗体（抗-SLA）、抗糖蛋白210抗体和抗核蛋白体100抗体；间接免疫荧光法检测抗核抗体（ANA）、抗线粒体抗体、抗平滑肌抗体（ASMA）、抗肝肾微粒体抗体（抗-LKM）等自身抗体。多组间数据比较采用单因素方差分析或秩和检验分析。 结果： 23 832例次自身抗体检测中，共检出38例抗-LC1阳性患者，初次诊断年龄11.0~84.0（50.6±16.0）岁；男性8例（21.1%），女性30例（78.9%）；抗-LC1与ANA共阳性31例（81.6%），核型以核颗粒型为主，占比为54.8%；与ASMA共阳性5例（13.2%），未见与抗-LKM或抗-SLA同时阳性者。38例抗-LC1阳性患者中，确诊自身免疫性肝炎（AIH）9例，可能AIH 6例，原发性胆汁性胆管炎（PBC）患者6例，乙型肝炎患者8例，丙型肝炎患者2例，酒精性肝病1例，非酒精性脂肪性肝病2例，药物性肝损伤1例，肝豆状核变性1例，肿瘤患者2例。确诊和可能AIH病例占抗-LC1阳性者的39.5%（15/38）。抗-LC1阳性者中已进入肝硬化期患者47.4%（18/38）。AIH组球蛋白水平高于HBV组（P = 0.006）和其他疾病组（P = 0.001）；AIH组IgG水平高于PBC组（P = 0.027）、HBV组（P = 0.009）和其他疾病组（P = 0.004）；PBC组IgM水平高于AIH组（P = 0.003）、HBV组（P = 0.003）和其他疾病组（P = 0.006）。 结论： 抗-LC1并非只在AIH中被检出，可以见于PBC、乙型及丙型肝炎、酒精性及非酒精性肝病、药物性肝损伤、遗传代谢性肝病和肿瘤患者中。以女性为主，多数伴ANA阳性，可见于青年和中老年患者，近半数患者已发展至肝硬化。抗-LC1是否是诊断2型自身免疫性肝炎特异性抗体需进一步研究，但如果高度怀疑AIH，此抗体可作为加分项。.
Keywords: Antibodies to liver cytosol type 1; Autoimmune hepatitis; Liver disease.