自身免疫性肝炎临床、免疫学及病理学特征分析142例

Clinical, immunological and pathological characterization of autoimmune hepatitis: an analysis of 142 cases

目的:了解自身免疫性肝炎(AIH)患者的临床、免疫学和病理学特点.方法:回顾性分析142例AIH患者的一般资料、临床特点、生化检查、免疫学检查和自身抗体的结果.结果:142例AIH患者男女比例1∶3.73,发病年龄为52.57±14.85岁,首次发病诊断分别为慢性肝炎(42.3%)、急性肝炎(34.5%)、重型肝炎(9.9%)和肝硬化(13.4%);临床表现主要为乏力(97.2%)、纳差(84.5%)、黄疸(84.5%)、发热(19.0%)、恶心(14.1%)和腹胀(12.7%)等.生化检查显示,ALT、AST、TBIL水平升高.AIH亚型中Ⅰ型多见(92.2%),Ⅱ型少见(6.3%),Ⅲ型罕见,仅1例.AIH重叠原发性胆汁性肝硬化(PBC)占14.1%,合并其他自身免疫性疾病占17.6%.免疫学检测显示,γ球蛋白和IgG升高明显.自身抗体检测出不同类型21种,以抗核抗体(82.4%),抗平滑肌抗体(19.7%),抗线粒体抗体(19%),抗SSA抗体(9.2%)多见.肝活检19例,均可见不同形式的肝细胞变性,坏死和浆细胞浸润,17例患者存在不同程度的纤维增生,1例有胆管轻度破坏.结论:AIH女性多见,发病和临床表现无特异性,生化检查以肝细胞损伤为主,Ⅰ型AIH最多见,检出自身抗体种类较多,但特异性和敏感性较高的自身抗体尚待进一步研究.肝组织学改变主要为不同程度的肝细胞变性,坏死,浆细胞浸润和纤维增生.胆管破坏少见.

AIM： To summarize the clinical, immunological and pathological features of autoimmune hepatitis （AIH）. METHODS： The data, including general information, clinical characteristics, and results of biochemical, immunological and pathological examinations, of 142 AIH patients were retrospectively analyzed. RESULTS： Most of AIH patients were women, with a mean age of 52.57 ± 14.85 years old, and the ratio of male to female was 1 ： 3.73. Of all the patients, 42.3% were presented with chronic hepatitis, 34.5% with acute hepatitis, 9.9% with severe hepatitis and 13.4% with cirrhosis. The main clinical manifestations included fatigue （97.2%）, inappetence （84.5%）, jaundice （84.5%）, fever （19%）, nausea （14.1%）, and abdominal swelling （12.7%）. AIH patients complicated with primary biliary cirrhosis covered a percentage of 14.1%, and those complicated with other autoimmune diseases covered 17.6%. Biochemical examination showed elevated levels of alanine aminotransferase, aspartate aminotransferase and total bilirubin. High level of y-globulin and immunoglobulin G were tested in most patients. The percentages of patients with Type 1 AIH and Type 2 AIH were 92.2% and 6.3%, respectively, while there was only 1 case with Type 3 AIH. A total of 21 kinds of auto-antibodies were detected, including ANA （72.4%）, SMA （19.7%）, AMA （19%）, anti-SSA （9.2%）, etc. Hepatocyte denaturation, necrosis and plasmocyte infiltration were demonstrated in all of the 19 patients received liver biopsy, and fibrosis was found in 17 cases and bile duct injury in 1 case. CONCLUSION： AIH occurred more frequently in females, and the onset and clinical features are not typical. Type 1 AIH is the most common one. There are many categories of antibodies in AIH, but the antibodies with higher specificity and sensibility should be further studied. Hepatocyte denaturation, necrosis, plasmocyte infiltration and fibrous proliferation are dominant histological features.