自身免疫性肝病的临床特点

Clinical features of autoimmune liver disease

目的:探讨自身免疫性肝病(ALD)的临床特征.方法:ALD患者91例,其中自身免疫性肝炎(AIH)68例,原发性胆汁性肝硬化(PBC)23例,另选同期住院的41例HBsAg阳性的慢性乙型肝炎肝硬化患者作为对照.分析所有患者的临床特点以及血液生化和血清免疫学检查结果.结果:AIH和PBC均以女性患者多见,发病年龄AIH为(51.2±13.4)岁,而PBC则是(52.8±10.3)岁,慢性乙型肝炎肝硬化患者则以男性多见,平均年龄为(57.1±13.0)岁.与慢性乙型肝炎肝硬化相比,AIH和PBC患者丙氨酸氨基转移酶(ALT)、碱性磷酸酶(ALP)、γ-谷氨酰基转移酶(GGT)、总蛋白(TP)、总胆红素(TBIL)均明显增高(P<0.05),PBC组的TBIL水平也明显高于AIH组(P<0.05).ALD患者胆红素升高以直接胆红素(DBIL)升高为主;三组白蛋白(ALB)均有降低,但以肝硬化组降低更显著.自身抗体的阳性率在AIH和PBC比较接近(分别为63.2%和78.3%),本组慢性乙型肝炎肝硬化病人自身抗体均为阴性.AIH组患者抗核抗体(ANA)、抗双链DNA抗体(抗-dsDNA)阳性率高于PBC组(P<0.05),PBC组患者抗线粒体抗体(AMA)阳性率高于AIH患者(P<0.05);自身抗体阴性的AIH组ALT较阳性组显著增高,自身抗体阴性的PBC组ALT、AST、ALP、GGT均低于阳性组(P<0.05).结论:ALD缺乏典型的临床特征.闭经前后的女性出现乏力和黄疸,结合γ球蛋白升高、病毒标志物阴性,高度怀疑ALD,自身抗体的出现是本病更好的佐证.单纯细胞酶(AST,ALT)的升高提示AIH,同时伴随胆道酶(ALP,GGT)和/或以DBIL升高为主的高胆红素血症,应高度怀疑PBC.

AIM：To investigate the clinical features of autoimmune liver disease（ALD）.METHODS：A total of 91 patients with ALD were enrolled in this study,including 68 cases of autoimmune hepatitis（AIH）and 23 cases of primary biliary cirrhosis（PBC）.Another 41 HBsAg-positive patients with cirrhosis were used as controls.Clinical manifestations,liver function parameters and serum immunoglobulins were compared among these patients.RESULTS：The median onset age for patients with AIH and PBC was 51.2±13.4 and 52.8± 10.3 years,respectively.Females were more likely to suffer from AIH and PBC than males.AIH patients had significantly higher levels of ALT,ALP,GGT,TP,and TBIL（all P〈0.05）than patients with chronic hepatitis B-related cirrhosis.Compared to patients with AIH,those with PBC had much higher levels of TBIL（P〈0.05） which were mainly caused by the increase in DBIL.All patients had decreased levels of ALB,especially those with chronic hepatitis B-related cirrhosis.Patients with AIH and those with PBC had nearly the same positive rate of autoantibodies（63.2%vs 78.3%）,while all patients with chronic hepatitis B-related cirrhosis were negative for autoantibodies.Patients with AIH had higher positive rates of ANA and anti-dsDNA but a lower positive rate of AMA than patients with PBC（all P〈0.05）.Autoantibody-negative AIH patients had significantly higher levels of ALT and AST,while autoantibody-negative PBC patients had lower levels of ALT,AST,ALP and GGT than autoantibody-positive ones.CONCLUSION：ALD lacks typical clinical features.Perimenopausal women who display fatigue and jaundice and have elevatedγ-globulin level and negative viral markers should be highly suspected of having ALD.The presence of autoantibodies may confirm the diagnosis.Patients with AIH often have abnormal transmainase levels,while high levels of transaminases activities（ALT,AST）and significantly increased biliary enzymes（ALP,GGT）and direct bilirubin levels may suggest PBC.